HemeOnc Flashcards Flashcards by Ryan Hutten

Typical presentation of Diffuse Large B-cell Lymphoma

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

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Typical presentation of Peripheral T-cell lymphoma NOS

Lymphadenopathy, eosinophilia, pruritis, fever and weight loss

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Relevant cell types and diagnosis of Hairy Cell Leukemia

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

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Typical presentation of Polycythemia vera

Massive increase in RBC mass, hematocrit near 60%

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Typical presentation of Adult T-cell Leukemia/Lymphoma

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

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Typical presentation of Monoclonal gammopathy of undetermined significance

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

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Typical presentation of Hodgkin Lymphoma

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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Relevant cell types and diagnosis of Polycythemia vera

Neoplastic cells: Erythroid precursors. Diagnosis: JAK2 mutation

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

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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Relevant cell types and diagnosis of Mantle Cell Lymphoma

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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Typical presentation of Essential Thrombocytosis

Patients present with thrombosis/hemorrhage due to platelet abnormalities

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Prognosis of Diffuse Large B-cell Lymphoma

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

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Prognosis of Burkitt Lymphoma

Aggressive,

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Prognosis of Hairy Cell Leukemia

Excellent prognosis, respond to gentle chemotherapy

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Prognosis of Chronic myelogenous leukemia

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

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Prognosis of Acute Lymphoblastic leukemia (ALL)

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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Relevant cell types and diagnosis of Chronic myelogenous leukemia

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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Prognosis of Hodgkin Lymphoma

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

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Typical presentation of Chronic myelogenous leukemia

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

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Relevant cell types and diagnosis of Hodgkin Lymphoma

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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Relevant cell types and diagnosis of Primary myelofibrosis

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

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

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

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

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

Relevant cell types and diagnosis of Follicular Lymphoma

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

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

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

Relevant cell types and diagnosis of Monoclonal gammopathy of undetermined significance

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

Typical presentation of Acute Myeloid leukemia (AML)

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

Prognosis of Adult T-cell Leukemia/Lymphoma

Usually fatal

Typical presentation of Multiple myeloma

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

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

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

Prognosis of Mantle Cell Lymphoma

Aggressive, median survival 3-5 years

Relevant cell types and diagnosis of Burkitt Lymphoma

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

Prognosis of Multiple myeloma

Recurrent infections, renal insufficiency, amyloidosis

Typical presentation of Acute Lymphoblastic leukemia (ALL)

80% are in children (\<15 yo)

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

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

Prognosis of Peripheral T-cell lymphoma NOS

No therapies currently available

Prognosis of Essential Thrombocytosis

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

Relevant cell types and diagnosis of Essential Thrombocytosis

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

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

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)

Typical presentation of Hairy Cell Leukemia

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

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

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

Typical presentation of Mantle Cell Lymphoma

4% of NHL's, mostly in older males

Prognosis of Polycythemia vera

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

Relevant cell types and diagnosis of Multiple myeloma

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

Prognosis of Acute Myeloid leukemia (AML)

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

Prognosis of Monoclonal gammopathy of undetermined significance

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

Prognosis of Follicular Lymphoma

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

Prognosis of Primary myelofibrosis

Median survival 1-5 years

Typical presentation of Primary myelofibrosis

Elderly patients presenting with progressive anemia, splenic enlargement

Typical presentation of Follicular Lymphoma

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

Typical presentation of Burkitt Lymphoma

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