HemeOnc Flashcards

1
Q

Typical presentation of Diffuse Large B-cell Lymphoma

A

Most common adult lymphoma, 50% of NHLs. Heterogenous presentation, usually involving a rapidly enlarging symptomatic mass

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2
Q

Typical presentation of Peripheral T-cell lymphoma NOS

A

Lymphadenopathy, eosinophilia, pruritis, fever and weight loss

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3
Q

Relevant cell types and diagnosis of Hairy Cell Leukemia

A

Neoplastic cells: Neoplastic B cells have hair-like cytoplasmic projections. Diagnosis: Cytochemical stain TRAP+

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4
Q

Typical presentation of Polycythemia vera

A

Massive increase in RBC mass, hematocrit near 60%

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5
Q

Typical presentation of Adult T-cell Leukemia/Lymphoma

A

Rare in US, but seen in Japan, West Africa and Caribbean. Patients present with skin lesions, hepatosplenomegaly, lymphocytosis and hypercalcemia.

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6
Q

Typical presentation of Monoclonal gammopathy of undetermined significance

A

Most common plasma cell dyscrasia in elderly (>50yo). Patients are asymptomatic

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7
Q

Typical presentation of Hodgkin Lymphoma

A

Bimodal age involvement (young adults, older adults) present with asymptomatic enlargement of one lymph node. Spreads to anatomically contiguous lymphoid tissue.

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8
Q

Relevant cell types and diagnosis of Polycythemia vera

A

Neoplastic cells: Erythroid precursors. Diagnosis: JAK2 mutation

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9
Q

Relevant cell types and diagnosis of Diffuse Large B-cell Lymphoma

A

Neoplastic cells: 2 types: Germinal center B-cell (GCB) and activated B-cell (ABC). Diagnosis: Most have B cell CD20 marker, chromosomal abnormalities detected with FISH: t(14;18), BCL6 rearrangement on 3q27, MYC translocation

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10
Q

Relevant cell types and diagnosis of Mantle Cell Lymphoma

A

Neoplastic cells: Small B cells with irregular nuclei, CD19, CD20 and CD5 positive. Diagnosis: t(11;14) causes CyclinD1-IGH fusion protein, increased proliferation

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11
Q

Typical presentation of Essential Thrombocytosis

A

Patients present with thrombosis/hemorrhage due to platelet abnormalities

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12
Q

Prognosis of Diffuse Large B-cell Lymphoma

A

Complete remission in 60-80% of patients. ABC type has worse prognosis than GCB type

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13
Q

Prognosis of Burkitt Lymphoma

A

Aggressive,

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14
Q

Prognosis of Hairy Cell Leukemia

A

Excellent prognosis, respond to gentle chemotherapy

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15
Q

Prognosis of Chronic myelogenous leukemia

A

Eventual transformation to blast crisis, which looks like AML. Chemotherapy can control stable phase, but not blast crisis

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16
Q

Prognosis of Acute Lymphoblastic leukemia (ALL)

A

90% remission rate, cure in 85%. Best prognosis is children between 2-10yo, hyperdiploid, t(12,21) translocation. Poor prognosis 10yo, t(9,22)=BCR-ABL

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17
Q

Relevant cell types and diagnosis of Chronic myelogenous leukemia

A

Neoplastic cells: Granulocytic precursors in the marrow. Diagnosis: Philadelphia chromosome in 90% of patients, BCR-ABL fusion protein leads to cell proliferation, marrow almost 100% cellular

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18
Q

Prognosis of Hodgkin Lymphoma

A

Depends on subtype. Lymphocyte rich has the best prognosis, lymphocyte-depleted is the most aggressive form

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19
Q

Typical presentation of Chronic myelogenous leukemia

A

Insidious onset with non-specific symptoms. Slowly progresses for 3 years before accelerated phase with anemia and thrombocytopenia

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20
Q

Relevant cell types and diagnosis of Hodgkin Lymphoma

A

Neoplastic cells: Reed-Sternberg cells are derived from clonal B cells, recruit reactive cells via cytokine release. Diagnosis: RS cells seen on biopsy

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21
Q

Relevant cell types and diagnosis of Primary myelofibrosis

A

Neoplastic cells: Excessive collagen deposition in bone marrow caused by excessive PDGF and TGF-beta secretion by neoplastic megakaryocytes. Diagnosis: JAK2 mutations (50-60%), CALR mutations (30-40%), MPL mutations rarely

22
Q

Relevant cell types and diagnosis of Adult T-cell Leukemia/Lymphoma

A

Neoplastic cells: CD4 T-cells in adults infected with HTLV-1. Diagnosis: Floret-like lymphocytes are seen in peripheral blood

23
Q

Prognosis of Chronic Lymphocytic Leukemia/ Small Lymphocytic Lymphoma (CLL/SLL)

A

Depends on involvement of IGH gene (IGH mutations = good prognosis)

24
Q

Relevant cell types and diagnosis of Follicular Lymphoma

A

Neoplastic cells: B cells: centrocytes and centroblasts that are CD19, CD20 and CD10 positive. Diagnosis: t(14;18) causes BCL2-IGH fusion protein, preventing apoptosis

25
Q

Relevant cell types and diagnosis of Chronic Lymphocytic Leukemia/ Small Lymphocytic Lymphoma (CLL/SLL)

A

Neoplastic cells: Mature B cell neoplasm (CD20, CD5, CD23). Diagnosis: Peripheral blood with small lymphocytes, smudge cells. Loss of lymph node structure

26
Q

Relevant cell types and diagnosis of Monoclonal gammopathy of undetermined significance

A

Neoplastic cells: Plasma cell proliferation, less than multiple myeloma. Diagnosis: M-spike on electrophoresis less than 3 gm/dl

27
Q

Typical presentation of Acute Myeloid leukemia (AML)

A

Adults >60yo, 20% of childhood leukemias. Heterogenous presentation.

28
Q

Prognosis of Adult T-cell Leukemia/Lymphoma

A

Usually fatal

29
Q

Typical presentation of Multiple myeloma

A

Median age: 70 yo; present with hypercalcemia, renal insufficiency, anemia, and bone lesion

30
Q

Relevant cell types and diagnosis of Peripheral T-cell lymphoma NOS

A

Neoplastic cells: T cell (CD3+ with loss of other pan-T cell markers). Diagnosis: No specific histologic feature

31
Q

Prognosis of Mantle Cell Lymphoma

A

Aggressive, median survival 3-5 years

32
Q

Relevant cell types and diagnosis of Burkitt Lymphoma

A

Neoplastic cells: B cell neoplasms positive for CD20, CD19 and CD10. Diagnosis: Round tumor cells of intermediate size in a starry sky pattern. Hx of EBV infection. t(8;14) causes MYC-IGH fusion protein, increased proliferation

33
Q

Prognosis of Multiple myeloma

A

Recurrent infections, renal insufficiency, amyloidosis

34
Q

Typical presentation of Acute Lymphoblastic leukemia (ALL)

A

80% are in children (<15 yo)

35
Q

Typical presentation of Chronic Lymphocytic Leukemia/ Small Lymphocytic Lymphoma (CLL/SLL)

A

Most common leukemia of adults, often in patients with immune dysfunction, hypoagammaglobulinemia

36
Q

Prognosis of Peripheral T-cell lymphoma NOS

A

No therapies currently available

37
Q

Prognosis of Essential Thrombocytosis

A

12-15 year median survival with low transformation rate. Treated with aspirin.

38
Q

Relevant cell types and diagnosis of Essential Thrombocytosis

A

Neoplastic cells: Neoplastic megakaryocytes and their lineage cells. Diagnosis: Activating point mutations in JAK2 (50%), CALR (40%), MPL (10%)

39
Q

Relevant cell types and diagnosis of Acute Lymphoblastic leukemia (ALL)

A

Neoplastic cells: Lymphoblasts: precursor B or T lymphocytes. 85% are B-ALL, 15% are T-ALL. Diagnosis: Stain TdT positive 95% of the time. 90% have structural chromosome abnormality (hyperdiploidy or translocation)

40
Q

Typical presentation of Hairy Cell Leukemia

A

Middle aged men present with pancytopenia, monocytopenia, spenomegaly, hepatomegaly or infections

41
Q

Relevant cell types and diagnosis of Acute Myeloid leukemia (AML)

A

Neoplastic cells: Myeloblasts from genetic abnormalities, myelodysplastic syndrome, therapy, or other (NOS). Hypercellular marrow produces ineffective cells, peripheral cytopenia. . Diagnosis: Myeloblasts seen in >20%. Stain myeloperoxidase positive, alpha napthyl butyrate esterase positive. Auer rods are seen

42
Q

Typical presentation of Mantle Cell Lymphoma

A

4% of NHL’s, mostly in older males

43
Q

Prognosis of Polycythemia vera

A

Survival >10 years. Thrombosis, bleeding can cause death if not treated. Can transition to myelofibrosis, AML

44
Q

Relevant cell types and diagnosis of Multiple myeloma

A

Neoplastic cells: Monoclonal plasma cell proliferation in the bone marrow. Diagnosis: M-protein in serum, urine (IgG usually, sometimes IgA). Lytic lesions seen on x-ray. Serum electrophoresis showing monoclonal Ig proliferation

45
Q

Prognosis of Acute Myeloid leukemia (AML)

A

Heterogenous prognosis. Good prognoses: t(15,17), t(8,21), inverted c16. Poor prognoses for 11q23. Chemo not very successful, bone marrow transplants important

46
Q

Prognosis of Monoclonal gammopathy of undetermined significance

A

1% of patients with MGUS develop symptomatic plasma cell neoplasm. No treatment, just monitor progression.

47
Q

Prognosis of Follicular Lymphoma

A

Median survival 7-9 years. Anti-CD20 therapy is effective. 30-50% transform to DLCL with poor prognosis

48
Q

Prognosis of Primary myelofibrosis

A

Median survival 1-5 years

49
Q

Typical presentation of Primary myelofibrosis

A

Elderly patients presenting with progressive anemia, splenic enlargement

50
Q

Typical presentation of Follicular Lymphoma

A

40% of adults NHL’s, adults present with painless lymphadenopathy

51
Q

Typical presentation of Burkitt Lymphoma

A

Children/young adults with tumors of mandible (endemic-African) or abdomen (non-endemic)