Path 13 Flashcards

During which embryonic week does primitive blood cell formation first begin?

A) First week

B) Third week

C) Sixth week

D) Tenth week

What is the earliest site of embryonic blood cell formation?

A) Fetal liver

B) Bone marrow

C) Spleen

D) Yolk sac

Which organ is the primary site of hematopoiesis during much of the second trimester?

A) Liver

B) Spleen

C) Bone marrow

D) Thymus

Which tissue becomes the primary site of hematopoiesis during late fetal development and after birth?

A) Yolk sac

B) Liver

C) Bone marrow

D) Spleen

After puberty, hematopoiesis primarily occurs in which skeletal regions?

A) Distal long bones

B) Axial and proximal appendicular skeleton

C) Hands and feet

D) Cartilaginous growth plates

All mature blood cell types ultimately arise from which cells?

A) Hematopoietic stem cells

B) Mature lymphocytes

C) Mesenchymal fibroblasts

D) Circulating monocytes

Which two properties characterize hematopoietic stem cells?

A) Unipotency and terminal differentiation

B) Pluripotency and limited division

C) Multipotency and self-renewal

D) Totipotency and senescence

Which vascular structures allow mature blood cells to enter circulation from bone marrow?

A) Continuous capillaries

B) Fenestrated arterioles

C) Muscular venules

D) Marrow sinusoids

Which structural feature of bone marrow sinusoids facilitates blood cell passage into circulation?

A) Thick continuous basement membrane

B) Discontinuous basement membrane

C) Keratinized endothelial lining

D) Dense epithelial tight junctions

What is the term for simultaneous appearance of immature granulocytes and nucleated erythrocytes in peripheral blood?

A) Leukoerythroblastosis

B) Agranulocytosis

C) Pancytopenia

D) Reticulocytosis

What is a common cause of acquired agranulocytosis?

A) Excess erythropoietin

B) Increased vitamin B12

C) Drug-induced marrow toxicity

D) Excess plasma iron

A patient develops agranulocytosis following chemotherapy. Which drug classes commonly cause this complication?

A) Antihistamines and antacids

B) Diuretics and beta blockers

C) Anticoagulants and antiplatelets

D) Alkylating agents and antimetabolites

A patient with hypersplenism develops peripheral cytopenias and compensatory marrow activity. Which marrow finding may be present?

A) Complete fatty replacement

B) Reactive hypercellularity

C) Absent hematopoietic precursors

D) Diffuse marrow necrosis

What is the characteristic bone marrow appearance in aplastic anemia?

A) Hypocellular with fatty replacement

B) Hypercellular with increased blasts

C) Hypercellular with megakaryocytes

D) Normal cellularity with fibrosis

Which cytokine primarily promotes eosinophil production and maturation?

A) IL-2

B) IL-4

C) IL-5

D) IL-12

A patient with severe bacterial sepsis has neutrophils containing Döhle bodies and toxic granulation. What do these findings indicate?

A) Chronic lymphocyte proliferation

B) Defective neutrophil adhesion

C) Malignant plasma cell transformation

D) Reactive changes from severe inflammation

Acute lymphadenitis most commonly develops in response to which stimulus?

A) Chronic venous obstruction

B) Microbial infection

C) Excess erythropoietin

D) Increased platelet destruction

Which lymph node change is associated with stimulation of humoral immune responses?

A) Follicular hyperplasia

B) Paracortical hyperplasia

C) Sinus histiocytosis

D) Medullary fibrosis

Follicular hyperplasia primarily reflects proliferation of which immune cell population?

A) Neutrophils

B) Cytotoxic T cells

C) B lymphocytes

D) Natural killer cells

What is the main function of tingible-body macrophages within reactive germinal centers?

A) Produce immunoglobulin antibodies

B) Activate complement proteins

C) Secrete erythropoietin

D) Remove apoptotic B cells

Which lymph node change is associated with T-cell-mediated immune activation?

A) Follicular hyperplasia

B) Paracortical hyperplasia

C) Sinus histiocytosis

D) Germinal center atrophy

A lymph node draining a breast carcinoma demonstrates increased macrophages within its sinuses. What is this finding called?

A) Sinus histiocytosis

B) Follicular hyperplasia

C) Paracortical hyperplasia

D) Granulomatous lymphadenitis

Hemophagocytic lymphohistiocytosis (HLH) involves excessive activation of which immune cells?

A) Eosinophils and basophils

B) Plasma cells and neutrophils

C) Macrophages and T lymphocytes

D) Erythrocytes and platelets

Which viral infection is a well-recognized trigger of hemophagocytic lymphohistiocytosis?

A) Rhinovirus

B) Rotavirus

C) Adenovirus

D) Epstein-Barr virus

Which laboratory pattern strongly suggests hemophagocytic lymphohistiocytosis in a patient with persistent fever and systemic inflammation?

A) Elevated platelets, decreased ferritin

B) Cytopenias, markedly elevated ferritin

C) Polycythemia, decreased liver enzymes

D) Thrombocytosis, reduced triglycerides

Which hematologic malignancy is strongly associated with HHV-8 infection?

A) Follicular lymphoma

B) Mantle cell lymphoma

C) Primary effusion lymphoma

D) Burkitt lymphoma

Chronic H. pylori infection is associated with which gastric malignancy?

A) Gastric MALT lymphoma

B) Diffuse large B-cell lymphoma

C) Mantle cell lymphoma

D) Follicular lymphoma

Which treatment may induce regression of H. pylori-associated gastric MALT lymphoma?

A) High-dose corticosteroids

B) Antiviral therapy

C) Bone marrow transplantation

D) H. pylori eradication

HIV infection increases the risk of developing which hematologic malignancy?

A) Chronic myeloid leukemia

B) B-cell non-Hodgkin lymphoma

C) Essential thrombocythemia

D) Polycythemia vera

Which oncogenic virus frequently contributes to HIV-associated B-cell lymphomas?

A) Epstein-Barr virus

B) Cytomegalovirus

C) Hepatitis A virus

D) JC polyomavirus

Exposure to benzene, including through cigarette smoke, increases the risk of which leukemia?

A) Chronic lymphocytic leukemia

B) Acute lymphoblastic leukemia

C) Acute myeloid leukemia

D) Chronic myeloid leukemia

What is the most common malignancy diagnosed in children?

A) Neuroblastoma

B) Acute myeloid leukemia

C) Hodgkin lymphoma

D) Acute lymphoblastic leukemia

Which gene is commonly mutated in T-cell acute lymphoblastic leukemia?

A) PAX5

B) NOTCH1

C) ETV6

D) RUNX1

Which group of genes is associated with B-cell acute lymphoblastic leukemia?

A) JAK2, CALR, MPL

B) MYD88, CXCR4, BRAF

C) PAX5, TCF3, ETV6, RUNX1

D) KIT, FLT3, CEBPA, NPM1

What is the typical bone marrow appearance in untreated ALL?

A) Hypercellular with numerous lymphoblasts

B) Hypocellular with fatty replacement

C) Normal cellularity with mature lymphocytes

D) Fibrotic with few hematopoietic cells

Which ALL subtype commonly presents with an anterior mediastinal thymic mass?

A) B-ALL

B) Burkitt lymphoma

C) CLL

D) T-ALL

Which additional clinical findings may occur in patients with T-ALL?

A) Isolated polycythemia

B) Lymphadenopathy and splenomegaly

C) Severe isolated thrombocytosis

D) Absence of lymphoid enlargement

What produces the starry-sky appearance sometimes seen in highly proliferative lymphoid malignancies?

A) Extensive marrow fibrosis

B) Increased neutrophil granules

C) Macrophages engulfing apoptotic tumor cells

D) Accumulation of plasma cells

Which hematologic malignancy is classically associated with a starry-sky histologic appearance?

A) Burkitt lymphoma

B) Chronic lymphocytic leukemia

C) Hodgkin lymphoma

D) Follicular lymphoma

Which cytoplasmic characteristic is commonly seen in lymphoblasts of ALL?

A) Abundant coarse granules

B) Numerous Auer rods

C) Prominent eosinophilic granules

D) Scant agranular cytoplasm

Which cytoplasmic finding strongly supports AML rather than ALL?

A) Absence of visible granules

B) Presence of Auer rods

C) Scant basophilic cytoplasm

D) High nuclear-cytoplasmic ratio

Which enzyme marker is commonly positive in AML but negative in ALL?

A) Terminal deoxynucleotidyl transferase

B) Alkaline phosphatase

C) Myeloperoxidase

D) Acid phosphatase

Which nuclear enzyme is commonly expressed by immature lymphoblasts in ALL?

A) Terminal deoxynucleotidyl transferase

B) Myeloperoxidase

C) Lactate dehydrogenase

D) Glucose-6-phosphate dehydrogenase

Which immunophenotypic pattern most strongly supports precursor ALL rather than typical AML?

A) MPO-positive, TdT-negative

B) CD14-positive, MPO-positive

C) CD33-positive, CD117-positive

D) TdT-positive, MPO-negative

Which cell-surface marker is commonly expressed in B-ALL?

A) CD3

B) CD19

C) CD14

D) CD56

Which marker is commonly expressed in CD10-positive precursor B-ALL?

A) CD16

B) CD8

C) CD10

D) CD14

Which immunophenotypic pattern is characteristic of early pro-B ALL?

A) CD19-positive, CD10-negative

B) CD3-positive, CD19-negative

C) CD14-positive, CD33-positive

D) CD20-positive, TdT-negative

Which marker is most specific for T-cell lineage in T-ALL?

A) CD19

B) CD20

C) CD33

D) Cytoplasmic CD3

Which clinical symptom may occur in children with ALL due to marrow infiltration?

A) Persistent hypertension

B) Bone pain

C) Isolated hematuria

D) Progressive hyperglycemia

Which chromosomal translocation is associated with favorable prognosis in childhood B-ALL?

A) t(9;22)

B) t(4;11)

C) t(12;21)

D) t(8;14)

The t(12;21) translocation in B-ALL produces which gene fusion?

A) ETV6-RUNX1

B) BCR-ABL1

C) PML-RARA

D) TCF3-PBX1

Which chromosomal translocation produces the Philadelphia chromosome in B-ALL?

A) t(8;14)

B) t(12;21)

C) t(15;17)

D) t(9;22)

Which gene fusion results from the Philadelphia chromosome in B-ALL?

A) PML-RARA

B) BCR-ABL1

C) ETV6-RUNX1

D) RUNX1-RUNX1T1

What is the most common adult leukemia in Western populations?

A) Acute myeloid leukemia

B) Chronic myeloid leukemia

C) Chronic lymphocytic leukemia

D) Acute lymphoblastic leukemia

Which molecular abnormality commonly contributes to CLL by increasing B-cell survival?

A) 13q14 deletion causing BCL2 upregulation

B) 9;22 translocation activating BCR-ABL1

C) 15;17 translocation producing PML-RARA

D) 8;14 translocation activating MYC

Which effect results from increased BCL2 expression in CLL cells?

A) Increased cellular differentiation

B) Decreased apoptosis

C) Increased DNA repair

D) Decreased lymphocyte survival

Which antiapoptotic protein is commonly overexpressed in CLL?

A) BCL6

B) MYC

C) BCL2

D) Cyclin D1

Inhibitors of which enzyme can produce sustained therapeutic responses in CLL?

A) Janus kinase 2

B) Dihydrofolate reductase

C) Tyrosine hydroxylase

D) Bruton tyrosine kinase

Which characteristic structures are commonly seen in lymph nodes affected by CLL?

A) Reed-Sternberg cells

B) Proliferation centers

C) Caseating granulomas

D) Auer rods

Which peripheral blood smear finding is characteristic of CLL?

A) Auer rods

B) Howell-Jolly bodies

C) Smudge cells

D) Döhle bodies

Why do smudge cells appear on peripheral blood smears in CLL?

A) Fragile lymphocytes rupture during preparation

B) Erythrocytes undergo complement-mediated lysis

C) Neutrophils release cytoplasmic granules

D) Platelets aggregate during staining

Besides bone marrow and lymph nodes, which organs are commonly involved in CLL?

A) Kidneys and pancreas

B) Heart and lungs

C) Brain and thyroid

D) Liver and spleen

Which immunophenotypic pattern is characteristic of CLL?

A) CD3+, CD4+, CD8+

B) CD5+, CD19+, CD23+

C) CD10+, CD15+, CD30+

D) CD14+, CD33+, CD117+

Which surface immunoglobulins are typically weakly expressed by CLL cells?

A) IgA and IgE

B) IgG and IgA

C) IgM and IgD

D) IgE and IgG

What is the recognized precursor condition of CLL?

A) Monoclonal B-cell lymphocytosis

B) Monoclonal gammopathy of undetermined significance

C) Myelodysplastic syndrome

D) Essential thrombocythemia

Why are patients with CLL particularly susceptible to recurrent bacterial infections?

A) Excessive neutrophil production

B) Increased complement activation

C) Excessive IgE production

D) Hypogammaglobulinemia

Which autoimmune complications can develop in patients with CLL?

A) Polycythemia and thrombocytosis

B) Hemolytic anemia and thrombocytopenia

C) Neutrophilia and eosinophilia

D) Aplastic anemia and leukocytosis

Transformation of CLL into an aggressive diffuse large B-cell lymphoma is called what?

A) Blast crisis

B) Leukemic conversion

C) Richter transformation

D) Myeloid transformation

Which clinical finding may suggest Richter transformation in a patient with CLL?

A) Rapidly enlarging lymph node

B) Stable mild lymphocytosis

C) Gradual reduction in spleen size

D) Isolated mild thrombocytopenia

Which chromosomal translocation is characteristic of follicular lymphoma?

A) t(8;14)

B) t(9;22)

C) t(11;14)

D) t(14;18)

The t(14;18) translocation in follicular lymphoma causes overexpression of which protein?

A) BCL6

B) BCL2

C) MYC

D) Cyclin D1

What is the main effect of BCL2 overexpression in follicular lymphoma?

A) Increased DNA replication

B) Increased cell-cycle progression

C) Inhibition of apoptosis

D) Increased antigen presentation

Which epigenetic regulatory gene is frequently mutated in follicular lymphoma?

A) KMT2D

B) NOTCH1

C) BCR

D) JAK2

KMT2D encodes an enzyme involved in which process?

A) DNA replication

B) Protein degradation

C) RNA splicing

D) Histone methylation

What is the characteristic lymph node growth pattern in follicular lymphoma?

A) Diffuse sheets of large cells

B) Nodular follicular growth

C) Scattered Reed-Sternberg cells

D) Extensive caseating granulomas

Which two cell types characterize follicular lymphoma histologically?

A) Myeloblasts and promyelocytes

B) Reed-Sternberg cells and eosinophils

C) Centrocytes and centroblasts

D) Plasma cells and macrophages

Which splenic region is commonly infiltrated by follicular lymphoma?

A) White pulp

B) Red pulp

C) Splenic sinuses

D) Splenic capsule

Which hepatic region may be involved by follicular lymphoma?

A) Central veins

B) Space of Disse

C) Hepatic sinusoids

D) Portal tracts

What is a common clinical presentation of follicular lymphoma?

A) Isolated bone pain

B) Generalized painless lymphadenopathy

C) Severe acute hemolysis

D) Profound neutrophilic leukocytosis

What is the most common type of non-Hodgkin lymphoma in adults?

A) Burkitt lymphoma

B) Mantle cell lymphoma

C) Diffuse large B-cell lymphoma

D) Follicular lymphoma

Which transcription factor is frequently dysregulated in diffuse large B-cell lymphoma?

A) BCL6

B) BCL2

C) Cyclin D1

D) NOTCH1

The BCL6 gene involved in DLBCL is located on which chromosome?

A) Chromosome 8

B) Chromosome 11

C) Chromosome 14

D) Chromosome 3

Which indolent lymphoma can transform into diffuse large B-cell lymphoma?

A) Burkitt lymphoma

B) Follicular lymphoma

C) Hodgkin lymphoma

D) T-lymphoblastic lymphoma

Which histologic appearance is characteristic of diffuse large B-cell lymphoma?

A) Small cells forming follicles

B) Spindle cells forming vascular spaces

C) Large cells with diffuse growth

D) Mature plasma cells forming sheets

Which nuclear finding is commonly seen in diffuse large B-cell lymphoma?

A) Prominent nucleoli

B) Absent nuclear chromatin

C) Segmented multilobed nuclei

D) Inconspicuous nucleoli

Which B-cell markers are commonly expressed by diffuse large B-cell lymphoma?

A) CD3 and CD5

B) CD15 and CD30

C) CD14 and CD33

D) CD19 and CD20

Which viral infection increases the risk of developing diffuse large B-cell lymphoma through immune dysfunction?

A) Hepatitis A

B) HIV

C) Rhinovirus

D) Rotavirus

Which virus is associated with a subset of HIV-related diffuse large B-cell lymphomas?

A) Human herpesvirus 6

B) Cytomegalovirus

C) Epstein-Barr virus

D) JC polyomavirus

Which oropharyngeal lymphoid structure can be involved by diffuse large B-cell lymphoma?

A) Waldeyer ring

B) Circle of Willis

C) Peyer patches

D) Hassall corpuscles

Which chromosomal translocation is characteristic of Burkitt lymphoma?

A) t(14;18)

B) t(9;22)

C) t(11;14)

D) t(8;14)

Which oncogene is activated by the characteristic translocation in Burkitt lymphoma?

A) BCL2

B) MYC

C) BCL6

D) CCND1

Which virus is strongly associated with endemic Burkitt lymphoma?

A) Cytomegalovirus

B) Human herpesvirus 8

C) Epstein-Barr virus

D) Hepatitis B virus

What causes the characteristic starry-sky appearance in Burkitt lymphoma?

A) Macrophages ingesting apoptotic tumor cells

B) Accumulation of mature plasma cells

C) Extensive extracellular amyloid deposition

D) Increased erythrocyte formation

Which cytoplasmic appearance is characteristic of Burkitt lymphoma cells on cytologic smears?

A) Pale cytoplasm without vacuoles

B) Abundant eosinophilic granules

C) Cytoplasm containing Auer rods

D) Deep-blue cytoplasm with vacuoles

Which anatomical site is classically involved in endemic Burkitt lymphoma?

A) Thyroid gland

B) Jaw

C) Kidney

D) Mediastinum

Which anatomical region is commonly involved in sporadic Burkitt lymphoma?

A) Thyroid gland

B) Anterior mediastinum

C) Ileocecal region

D) Salivary glands

Which translocation is characteristic of mantle cell lymphoma?

A) t(11;14)

B) t(8;14)

C) t(14;18)

D) t(9;22)

Which cell-cycle regulatory protein is overexpressed in mantle cell lymphoma?

A) BCL2

B) BCL6

C) MYC

D) Cyclin D1

Cyclin D1 promotes progression through which phase transition of the cell cycle?

A) G2 to M

B) G1 to S

C) M to G1

D) S to G2

Which lymphoma can present with lymphomatoid polyposis involving the colon?

A) Follicular lymphoma

B) Burkitt lymphoma

C) Mantle cell lymphoma

D) Hodgkin lymphoma

Which lymphoma typically contains a relatively uniform population of cells with irregular nuclear contours?

A) Burkitt lymphoma

B) Follicular lymphoma

C) Mantle cell lymphoma

D) Hodgkin lymphoma

Which lymphoma characteristically contains both centrocytes and centroblasts in neoplastic follicles?

A) Follicular lymphoma

B) Mantle cell lymphoma

C) Hairy cell leukemia

D) Marginal zone lymphoma

Which immunophenotypic pattern is characteristic of mantle cell lymphoma?

A) CD5−

B) CD3−

C) CD3+

D) CD5+

Which B-cell markers are typically expressed in mantle cell lymphoma?

A) CD3 and CD4

B) CD19 and CD20

C) CD15 and CD30

D) CD14 and CD33

Which marker helps distinguish mantle cell lymphoma from CLL when both express CD5?

A) CD19 positivity

B) CD20 positivity

C) Cyclin D1 positivity

D) Surface IgM expression

What is a common clinical presentation of mantle cell lymphoma?

A) Painless lymphadenopathy

B) Isolated severe anemia

C) Acute bone pain

D) Diffuse skin erythroderma

Which lymphoma can develop from chronic antigenic stimulation and sometimes regress after treating the underlying infection?

A) Mantle cell lymphoma

B) Burkitt lymphoma

C) Marginal zone lymphoma

D) Anaplastic large-cell lymphoma

Eradication of H. pylori can induce regression of which lymphoma?

A) Gastric MALT lymphoma

B) Mantle cell lymphoma

C) Burkitt lymphoma

D) Follicular lymphoma

Which hematologic malignancy is strongly associated with the BRAF V600E mutation?

A) Mantle cell lymphoma

B) Follicular lymphoma

C) Chronic lymphocytic leukemia

D) Hairy cell leukemia

Which peripheral blood finding is characteristic of hairy cell leukemia?

A) Prominent Auer rods

B) Fine cytoplasmic projections

C) Large Reed-Sternberg cells

D) Numerous nucleated erythrocytes

Which splenic compartment is predominantly infiltrated in hairy cell leukemia?

A) White pulp

B) Germinal centers

C) Red pulp

D) Splenic capsule

What happens to the splenic white pulp in hairy cell leukemia?

A) Becomes markedly atrophic

B) Develops reactive hyperplasia

C) Forms large germinal centers

D) Undergoes extensive calcification

What is a common physical examination finding in hairy cell leukemia?

A) Generalized erythroderma

B) Cervical lymphadenopathy

C) Thyroid enlargement

D) Marked splenomegaly

How does classic hairy cell leukemia generally respond to modern therapy?

A) Poorly, with immediate relapse

B) Favorably, with prolonged remissions

C) Poorly, despite complete resection

D) Favorably, without any treatment

Which immunophenotypic markers commonly identify mature peripheral T-cell lymphomas?

A) CD19, CD20, CD23

B) CD15, CD20, CD30

C) CD2, CD3, CD5

D) CD14, CD33, CD117

Peripheral T-cell lymphomas may additionally express which markers depending on their cellular subtype?

A) CD4 or CD8

B) CD19 or CD20

C) CD14 or CD33

D) CD15 or CD30

Which gene is rearranged in ALK-positive anaplastic large-cell lymphoma?

A) BCL2

B) MYC

C) CCND1

D) ALK

The ALK gene involved in anaplastic large-cell lymphoma is located on which chromosome?

A) Chromosome 8

B) Chromosome 2

C) Chromosome 11

D) Chromosome 14

Which nuclear morphology is characteristic of anaplastic large-cell lymphoma?

A) Flower-shaped nuclei

B) Cerebriform nuclei

C) Horseshoe-shaped nuclei

D) Segmented neutrophil nuclei

Which immunophenotypic marker is strongly expressed in anaplastic large-cell lymphoma?

A) CD30

B) CD19

C) CD10

D) CD23

ALK-positive anaplastic large-cell lymphoma is particularly common in which age group?

A) Elderly adults

B) Middle-aged adults

C) Newborn infants

D) Children and young adults

Which virus causes adult T-cell leukemia/lymphoma?

A) Epstein-Barr virus

B) HTLV-1

C) Human herpesvirus 8

D) Cytomegalovirus

Adult T-cell leukemia/lymphoma primarily involves malignant proliferation of which cells?

A) CD8+ cytotoxic T cells

B) CD19+ B lymphocytes

C) CD4+ helper T cells

D) CD56+ NK cells

Which peripheral blood morphology is characteristic of adult T-cell leukemia/lymphoma?

A) Cloverleaf-shaped nuclei

B) Horseshoe-shaped nuclei

C) Cerebriform nuclei

D) Segmented nuclei

What is the typical prognosis of aggressive adult T-cell leukemia/lymphoma?

A) Excellent without treatment

B) Favorable with observation

C) Indolent with lifelong remission

D) Poor with rapid progression

Mycosis fungoides and Sézary syndrome are malignancies primarily involving which lymphocyte population?

A) CD8+ cytotoxic T cells

B) CD4+ helper T cells

C) CD19+ B lymphocytes

D) CD56+ NK cells

Which organ is primarily affected in mycosis fungoides and Sézary syndrome?

A) Kidney

B) Liver

C) Skin

D) Spleen

Which nuclear morphology is characteristic of Sézary cells?

A) Cerebriform nuclei

B) Horseshoe-shaped nuclei

C) Cloverleaf-shaped nuclei

D) Multilobed neutrophil nuclei

Which clinical finding is particularly characteristic of Sézary syndrome?

A) Painless bone lesions

B) Massive splenomegaly alone

C) Isolated cervical lymphadenopathy

D) Generalized exfoliative erythroderma

Which gene is frequently mutated in T-cell large granular lymphocytic leukemia?

A) BRAF

B) STAT3

C) NOTCH1

D) ALK

T-cell large granular lymphocytic leukemia is often associated with which autoimmune disorder?

A) Systemic sclerosis

B) Graves disease

C) Rheumatoid arthritis

D) Myasthenia gravis

What three findings characterize Felty syndrome?

A) Arthritis, splenomegaly, neutropenia

B) Anemia, jaundice, thrombocytosis

C) Eczema, thrombocytopenia, infections

D) Hypercalcemia, renal failure, bone lesions

Which lymphoma classically exhibits angioinvasion and extensive ischemic tissue necrosis?

A) Follicular lymphoma

B) Mantle cell lymphoma

C) Marginal zone lymphoma

D) Extranodal NK/T-cell lymphoma

Which viral infection is strongly associated with extranodal NK/T-cell lymphoma?

A) HTLV-1

B) Epstein-Barr virus

C) Human herpesvirus 8

D) Hepatitis B virus

What cytoplasmic feature can be seen in malignant NK cells of extranodal NK/T-cell lymphoma?

A) Auer rods

B) Russell bodies

C) Azurophilic granules

D) Large lipid vacuoles

What do neoplastic plasma cells commonly produce in plasma cell disorders?

A) Monoclonal immunoglobulins or light chains

B) Polyclonal T-cell receptors

C) Complement membrane attack proteins

D) Erythrocyte surface antigens

Which plasma cell malignancy commonly produces multiple osteolytic lesions throughout the skeleton?

A) Waldenström macroglobulinemia

B) Hairy cell leukemia

C) Mantle cell lymphoma

D) Multiple myeloma

Which plasma cell or lymphoplasmacytic disorder is associated with elevated IgM and hyperviscosity?

A) Multiple myeloma

B) Waldenström macroglobulinemia

C) AL amyloidosis

D) Solitary plasmacytoma

Which combination of findings is characteristic of symptomatic multiple myeloma?

A) Erythroderma, lymphadenopathy, pruritus

B) Neutrophilia, thrombocytosis, splenomegaly

C) Hypercalcemia, renal dysfunction, lytic lesions

D) Thrombocytopenia, eczema, recurrent infections

Which cytokine supports plasma cell growth and is associated with multiple myeloma progression?

A) IL-6

B) IL-2

C) IL-5

D) IL-12

Which skeletal region is particularly commonly affected by osteolytic lesions in multiple myeloma?

A) Distal phalanges

B) Carpal bones

C) Patella

D) Vertebral column

Which lymphoma is more likely to involve Waldeyer's ring and mesenteric lymph nodes?

A) Classical Hodgkin lymphoma

B) Nodular sclerosis Hodgkin lymphoma

C) Non-Hodgkin lymphoma

D) Lymphocyte-rich Hodgkin lymphoma

Which lymphoma is more likely to present with extranodal involvement?

A) Non-Hodgkin lymphoma

B) Classical Hodgkin lymphoma

C) Nodular sclerosis Hodgkin lymphoma

D) Mixed-cellularity Hodgkin lymphoma

Which characteristic malignant cells are associated with classical Hodgkin lymphoma?

A) Centroblasts

B) Myeloblasts

C) Sézary cells

D) Reed-Sternberg cells

Reed-Sternberg cells originate from which cell lineage?

A) Myeloid precursor cells

B) Germinal center B cells

C) Mature T lymphocytes

D) Natural killer cells

Which transcription factor promotes Reed-Sternberg cell survival and proliferation?

A) MYC

B) STAT5

C) NF-κB

D) RUNX1

Which virus is associated with a subset of classical Hodgkin lymphomas?

A) Epstein-Barr virus

B) Human herpesvirus 8

C) HTLV-1

D) JC polyomavirus

Which immunophenotypic pattern is characteristic of classical Reed-Sternberg cells?

A) CD20+, CD10+, CD15−

B) CD3+, CD5+, CD30−

C) CD19+, CD20+, CD23+

D) CD15+, CD30+, weak PAX5+

What is the most common subtype of classical Hodgkin lymphoma?

A) Mixed cellularity

B) Nodular sclerosis

C) Lymphocyte depletion

D) Lymphocyte-rich

Which Hodgkin lymphoma subtype characteristically contains lacunar-type Reed-Sternberg cells?

A) Mixed cellularity

B) Lymphocyte depletion

C) Nodular sclerosis

D) Lymphocyte-rich

Which histologic finding is characteristic of nodular sclerosis Hodgkin lymphoma?

A) Broad collagen bands forming nodules

B) Diffuse sheets of myeloblasts

C) Numerous centrocytes forming follicles

D) Extensive vascular spindle-cell proliferation

Which lymph-node regions are commonly involved in nodular sclerosis Hodgkin lymphoma?

A) Mesenteric and inguinal

B) Iliac and para-aortic

C) Axillary and popliteal

D) Cervical, supraclavicular, and mediastinal

Which Hodgkin lymphoma subtype contains Reed-Sternberg cells within a mixed inflammatory background?

A) Nodular sclerosis

B) Mixed cellularity

C) Lymphocyte depletion

D) Lymphocyte-rich

Which classical Hodgkin lymphoma subtype is frequently associated with EBV and a heterogeneous inflammatory infiltrate?

A) Nodular sclerosis

B) Lymphocyte-rich

C) Mixed cellularity

D) Nodular lymphocyte-predominant

Which classical Hodgkin lymphoma subtype contains numerous reactive lymphocytes and relatively few Reed-Sternberg cells?

A) Lymphocyte-rich

B) Mixed cellularity

C) Lymphocyte depletion

D) Nodular sclerosis

Which Hodgkin lymphoma subtype contains abundant Reed-Sternberg cells but relatively few background lymphocytes?

A) Nodular sclerosis

B) Mixed cellularity

C) Lymphocyte-rich

D) Lymphocyte depletion

Which lymphoma is characterized by popcorn-shaped lymphocyte-predominant cells?

A) Nodular sclerosis Hodgkin lymphoma

B) Nodular lymphocyte-predominant lymphoma

C) Mixed-cellularity Hodgkin lymphoma

D) Lymphocyte-depleted Hodgkin lymphoma

Which lymphoma is usually EBV-negative and contains popcorn-shaped cells rather than classic Reed-Sternberg cells?

A) Nodular lymphocyte-predominant lymphoma

B) Mixed-cellularity Hodgkin lymphoma

C) Lymphocyte-depleted Hodgkin lymphoma

D) Nodular sclerosis Hodgkin lymphoma

Which Hodgkin lymphoma subtype commonly presents with localized stage I or II disease?

A) Lymphocyte depletion

B) Mixed cellularity

C) Advanced classical Hodgkin lymphoma

D) Nodular sclerosis

Which classical Hodgkin lymphoma subtype more frequently presents with advanced-stage disease?

A) Lymphocyte-rich

B) Lymphocyte depletion

C) Nodular sclerosis

D) Nodular lymphocyte-predominant

Which immune checkpoint ligands are frequently overexpressed in classical Hodgkin lymphoma?

A) CD19 and CD20

B) CD5 and CD23

C) PD-L1 and PD-L2

D) CD10 and BCL6

Which immune checkpoint inhibitor can be used to treat certain patients with classical Hodgkin lymphoma?

A) Pembrolizumab

B) Rituximab

C) Imatinib

D) Cytarabine

Which hematologic malignancy is characterized by proliferation of immature myeloid blasts?

A) Acute lymphoblastic leukemia

B) Chronic lymphocytic leukemia

C) Follicular lymphoma

D) Acute myeloid leukemia

Bone marrow replacement by leukemic blasts in AML commonly produces which abnormalities?

A) Polycythemia, thrombocytosis, neutrophilia

B) Anemia, thrombocytopenia, neutropenia

C) Erythrocytosis, leukocytosis, thrombocytosis

D) Hypercalcemia, hypernatremia, hyperkalemia

Which chromosomal translocation produces the RUNX1-RUNX1T1 fusion in AML?

A) t(15;17)

B) t(9;22)

C) t(8;21)

D) t(14;18)

Which chromosomal abnormality produces the CBFB-MYH11 fusion in AML?

A) inv(16)

B) inv(3)

C) t(11;14)

D) t(15;17)

AML with t(8;21) or inv(16) is classified under which genetic category?

A) KMT2A-rearranged AML

B) Therapy-related AML

C) NPM1-mutated AML

D) Core-binding factor AML

Which prognosis is generally associated with core-binding factor AML?

A) Uniformly adverse

B) Favorable

C) Uniformly intermediate

D) Universally fatal

Which chromosomal translocation is characteristic of acute promyelocytic leukemia?

A) t(8;21)

B) t(9;22)

C) t(15;17)

D) t(11;14)

Which fusion protein is produced by the characteristic translocation in acute promyelocytic leukemia?

A) PML-RARα

B) BCR-ABL1

C) RUNX1-RUNX1T1

D) CBFB-MYH11

With appropriate modern treatment, acute promyelocytic leukemia generally has which prognosis?

A) Uniformly fatal

B) Consistently poor

C) Untreatable

D) Highly favorable

Which AML-associated gene on chromosome 11q23 is frequently involved in rearrangements that can confer adverse risk?

A) RUNX1

B) KMT2A

C) CBFB

D) PML

AML is strongly associated with _____ syndrome in young children.

Which hematologic malignancy is characteristically associated with Auer rods?

A) Acute lymphoblastic leukemia

B) Chronic lymphocytic leukemia

C) Acute myeloid leukemia

D) Chronic myeloid leukemia

Which type of leukemic blast typically lacks Auer rods and stains positive for nonspecific esterase?

A) Myeloblast

B) Monoblast

C) Promyelocyte

D) Megakaryoblast

Which CD marker is commonly used to identify hematopoietic stem and progenitor cells?

A) CD34

B) CD15

C) CD20

D) CD3

Which CD marker is commonly expressed by immature myeloid cells?

A) CD19

B) CD15

C) CD3

D) CD33

Which CD marker is commonly expressed by mature granulocytic cells?

A) CD34

B) CD117

C) CD15

D) CD19

Therapy-related AML following topoisomerase II inhibitor exposure is associated with rearrangements involving which gene?

A) JAK2

B) KMT2A

C) BCR

D) BRAF

Therapy-related AML following alkylating chemotherapy is commonly associated with which chromosomal abnormalities?

A) Chromosome 5 and 7 losses

B) Chromosome 8 and 14 translocations

C) Chromosome 9 and 22 translocations

D) Chromosome 15 and 17 translocations

Which combination of peripheral blood abnormalities commonly occurs in AML due to marrow replacement?

A) Polycythemia, thrombocytosis, neutrophilia

B) Anemia, thrombocytosis, eosinophilia

C) Polycythemia, leukocytosis, basophilia

D) Anemia, thrombocytopenia, neutropenia

Which tissue is particularly susceptible to infiltration by AML with monocytic differentiation?

A) Thyroid gland

B) Pancreatic islets

C) Gingiva

D) Adrenal cortex

Which disorder involves ineffective hematopoiesis, dysplastic blood cell maturation, and increased AML risk?

A) Polycythemia vera

B) Myelodysplastic syndrome

C) Essential thrombocythemia

D) Chronic lymphocytic leukemia

Which chromosomal abnormality commonly occurs in myeloid neoplasms and increases copies of the MYC-containing chromosome?

A) Trisomy 8

B) Trisomy 13

C) Trisomy 18

D) Trisomy 21

Which erythroid abnormality can be seen in myelodysplastic syndromes?

A) Howell-Jolly bodies

B) Sickle-shaped erythrocytes

C) Increased reticulocytes

D) Ring sideroblasts

Ring sideroblasts contain iron-laden mitochondria arranged around which cellular structure?

A) Plasma membrane

B) Golgi apparatus

C) Nucleus

D) Endoplasmic reticulum

Which stain demonstrates the abnormal iron accumulation in ring sideroblasts?

A) Congo red

B) Prussian blue

C) Wright-Giemsa

D) Periodic acid-Schiff

Erythroid precursors in myelodysplastic syndrome may show morphological changes resembling which anemia?

A) Megaloblastic anemia

B) Hemolytic anemia

C) Iron deficiency anemia

D) Aplastic anemia

Which neutrophil abnormality is characteristic of myelodysplastic syndromes?

A) Hypersegmented nuclei exclusively

B) Toxic granulation exclusively

C) Increased cytoplasmic basophilia

D) Pseudo-Pelger-Huët anomaly

Pseudo-Pelger-Huët cells exhibit which nuclear abnormality?

A) Excessive nuclear segmentation

B) Multiple prominent nucleoli

C) Reduced nuclear segmentation

D) Complete nuclear absence

Which megakaryocyte abnormality may occur in myelodysplastic syndromes?

A) Giant multilobulated nuclei

B) Small hypolobated megakaryocytes

C) Complete absence of megakaryocytes

D) Normal megakaryocyte maturation

Primary myelodysplastic syndromes most commonly affect which population?

A) Older adults

B) Young children

C) Newborn infants

D) Adolescents

Which signaling pathway is frequently dysregulated in myeloproliferative neoplasms?

A) Wnt-beta-catenin signaling

B) Hedgehog signaling

C) TGF-beta signaling

D) JAK-STAT signaling

Which clinical and laboratory combination strongly suggests chronic myeloid leukemia?

A) Severe leukocytosis and splenomegaly

B) Isolated anemia and lymphopenia

C) Thrombocytopenia and hypogammaglobulinemia

D) Pancytopenia and absent marrow cells

Which additional bone marrow finding can occur in CML due to increased cellular turnover?

A) Numerous Reed-Sternberg cells

B) Extensive ring sideroblast formation

C) Prominent Auer rods

D) Sea-blue histiocytes

Which phase of CML is characterized by aggressive transformation with increased immature blasts?

A) Chronic phase

B) Latent phase

C) Blast crisis

D) Remission phase

Which gene is mutated in nearly all cases of polycythemia vera?

A) CALR

B) JAK2

C) BCR

D) BRAF

Which laboratory pattern is most characteristic of polycythemia vera?

A) Decreased hematocrit, increased erythropoietin

B) Increased hematocrit, increased erythropoietin

C) Decreased hematocrit, decreased erythropoietin

D) Increased hematocrit, decreased erythropoietin

Why is erythropoietin typically decreased in polycythemia vera?

A) Increased renal erythropoietin degradation

B) Complete destruction of renal tissue

C) Feedback suppression from erythrocytosis

D) Direct inhibition by platelets

Which vascular complication is a major cause of morbidity and mortality in polycythemia vera?

A) Arterial aneurysm

B) Thrombosis

C) Vasculitis

D) Capillary rupture

Which hepatic vascular complication can result from thrombosis in polycythemia vera?

A) Budd-Chiari syndrome

B) Gilbert syndrome

C) Wilson disease

D) Dubin-Johnson syndrome

Which gene mutations are commonly associated with essential thrombocythemia?

A) BCR, ABL1, PML

B) MYC, BCL2, BCL6

C) BRAF, NRAS, ALK

D) JAK2, CALR, MPL

Which blood cell population is predominantly increased in essential thrombocythemia?

A) Erythrocytes

B) Neutrophils

C) Platelets

D) Lymphocytes

Which bone marrow cell population is characteristically increased in essential thrombocythemia?

A) Erythroblasts

B) Megakaryocytes

C) Lymphoblasts

D) Plasma cells

Which vascular complication is associated with essential thrombocythemia?

A) Arterial and venous thrombosis

B) Isolated aortic aneurysms

C) Pulmonary arterial vasculitis

D) Congenital vascular malformations

Which symptom involves episodic burning pain and redness of the hands and feet in polycythemia vera or essential thrombocythemia?

A) Raynaud phenomenon

B) Acrocyanosis

C) Livedo reticularis

D) Erythromelalgia

Which disorder is characterized by progressive bone marrow fibrosis and abnormal megakaryocyte proliferation?

A) Polycythemia vera

B) Essential thrombocythemia

C) Primary myelofibrosis

D) Chronic lymphocytic leukemia

Which megakaryocyte morphology is characteristic of primary myelofibrosis?

A) Normal multilobulated nuclei

B) Cloud-like abnormal nuclei

C) Complete nuclear absence

D) Uniformly segmented nuclei

Which peripheral blood finding strongly suggests advanced primary myelofibrosis?

A) Teardrop erythrocytes

B) Target cells

C) Sickle cells

D) Spherocytes

Which genetic mutation is frequently found in Langerhans cell histiocytosis?

A) JAK2 V617F

B) MPL W515L

C) KIT D816V

D) BRAF V600E

Which cytoplasmic organelles are characteristic of Langerhans cell histiocytosis on electron microscopy?

A) Auer rods

B) Russell bodies

C) Birbeck granules

D) Döhle bodies

Which immunophenotypic markers are characteristic of Langerhans cell histiocytosis?

A) CD3 and CD5

B) CD1a and S100

C) CD19 and CD20

D) CD15 and CD30

A young child develops persistent seborrheic-like scalp lesions and a destructive bone lesion. Which diagnosis should be considered?

A) Langerhans cell histiocytosis

B) Chronic myeloid leukemia

C) Essential thrombocythemia

D) Polycythemia vera

What is the most common underlying cause of congestive splenomegaly?

A) Chronic pancreatitis

B) Renal artery stenosis

C) Pulmonary hypertension

D) Liver cirrhosis

Which embryological structure primarily gives rise to the thymus?

A) Second pharyngeal pouch

B) Fourth pharyngeal pouch

C) Third pharyngeal pouch

D) First pharyngeal pouch

Which immunodeficiency syndrome is associated with thymic aplasia or hypoplasia?

A) Wiskott-Aldrich syndrome

B) DiGeorge syndrome

C) Bruton agammaglobulinemia

D) Chediak-Higashi syndrome

Which autoimmune disorder is strongly associated with thymoma?

A) Myasthenia gravis

B) Multiple sclerosis

C) Rheumatoid arthritis

D) Systemic sclerosis