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Kristine Krafts, M.D.Chronic Leukemia, Lymphoma, and Myeloma
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Leukemias• Acute leukemias• Chronic leukemias
Lymphomas• Hodgkin lymphoma• Non-Hodgkin lymphoma
Plasma cell disorders• Multiple myeloma
Hematologic Malignancies
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Leukemias• Acute leukemias• Chronic leukemias
Hematologic Malignancies
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Chronic myeloid leukemia Chronic lymphocytic leukemia
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• Malignant proliferation of myeloid cells (not blasts, but maturing cells) in blood, bone marrow
• Four disorders; CML most important
• Occur only in adults
• Long course
Chronic Myeloproliferative Disorders
Things You Must Know
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• Neutrophilic leukocytosis
• Basophilia
• Philadelphia chromosome
• Three phases
Chronic Myeloid Leukemia
Things You Must Know
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Chronic Myeloid Leukemia
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Chronic Myeloid Leukemia
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All cases of CML have the Philadelphia chromosome!
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All cases of CML have the Philadelphia chromosome!
Normal chromosomes Chromosomes in CML
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ABL gene
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BCR gene
t(9;22)
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BCR-ABLhybrid geneABL gene
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All cases of CML have the Philadelphia chromosome!
t(9;22)
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BCR
ABL
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BCR-ABL fusion gene
NASTYtyrosine kinase
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Chronic phase• Stable counts (Hgb, WBC, platelets)• 3-4 years (without therapy)
Accelerated phase• Unstable counts• Fatal within months
Blast crisis• Lots of blasts (basically, acute leukemia!)• Fatal within weeks
Phases of CML
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Treatment• Used to be bone marrow transplant• Now, imatinib (Gleevec)
Prognosis• Median survival used to be 5-6 years• Now: ??
Treatment and Prognosis of CML
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Polycythemia vera• Tons of red cells in marrow, blood• Must rule out benign polycythemia!
Essential thrombocythemia• Tons of platelets in marrow, blood• Must rule out benign thrombocytosis!
Chronic myelofibrosis• Marrow is fibrotic; blood has teardrop red cells• Patients have extramedullary hematopoiesis
Other Myeloproliferative Disorders
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Chronic myelofibrosis: fibrotic marrow
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Chronic myelofibrosis: teardrop red cells in blood
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Polycythemia vera: ↑ ↑ ↑ red cells in blood
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Essential thrombocythemia: ↑ ↑ ↑ platelets in blood
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• Malignant proliferation of lymphocytes in blood, bone marrow
• Many disorders; CLL most important
• Occur only in adults
• Long course; indolent but incurable
Chronic Lymphoproliferative Disorders
Things You Must Know
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• Most common leukemia in adults
• Small, mature B lymphocytes
• Weird immunophenotype: CD5+
• Decreased normal immunoglobulins
Chronic Lymphocytic Leukemia
Things You Must Know
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CLL: tons of mature-appearing lymphocytes in blood
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CLL: tumor aggregates in marrow
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Leukemias• Acute leukemias• Chronic leukemias
Lymphomas• Hodgkin lymphoma• Non-Hodgkin lymphoma
Hematologic Malignancies
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• Malignant proliferation of lymphocytes that starts in lymph nodes
• Skips around• Many different types• Prognosis depends on specific type
Things you must know
Non-Hodgkin Lymphoma
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• Painless, firm lymphadenopathy• Extranodal manifestations• “B” symptoms: weight loss,
night sweats, fever
Symptoms of NHL
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• Most common cause overall: benign reaction to infection
• Most common malignant cause: metastatic carcinoma
Causes of lymphadenopathy
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Composed of small, mature, slowly-growing cells.
Low-grade lymphomas High-grade lymphomasComposed of big, ugly,
aggressive cells.
• Follicular lymphoma• MALT lymphoma• Mycosis fungoides
• Large-cell lymphoma• Burkitt lymphoma
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• Follicular pattern • “Butt” cells in blood• Grading is important for prognosis!• t(14;18)
Follicular Lymphoma
Things you must know
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Follicular lymphoma: back to back follicles
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Follicular lymphoma: three grades
Grade I Grade II Grade III
Mostly small cells
Relatively good prognosis
Small and large cells Mostly large cells
Relatively poorprognosis
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Follicular lymphoma: “butt” cells in blood
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Stage I
Stage II
Stage III
Stage IV
Single node
Two or more nodes onsame side of diaphragm
Lymph nodes on both sidesof the diaphragm
Diffuse extranodalinvolvement
90% 5ys
A = no additional symptomsB = weight loss, night sweats, fever
40% 5ys
Staging and Prognosis of Follicular Lymphoma
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• Occurs in mucosa-associated lymphoid tissue• Associated with Helicobacter pylori• Early on, can be cured with antibiotics
Things you must know
MALT Lymphoma
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• Skin lesions• Blood involvement• Cerebriform lymphocytes• T-cell immunophenotype
Things you must know
Mycosis Fungoides/Sézary Syndrome
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Raised, nodular skin lesions
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Pautrier microabscesses
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Cerebriform lymphocytes
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• Most common type of NHL• Large B cells• Extranodal involvement• Bad prognosis
Things you must know
Diffuse Large-Cell Lymphoma
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“Diffuse” pattern = just sheets of cells, no follicles
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Large lymphoma cells
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• Children, young adults• Often presents with extranodal mass• Fast-growing and aggressive• Starry-sky pattern
Things you must know
Burkitt Lymphoma
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Burkitt lymphoma
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Starry-sky pattern
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Burkitt lymphoma
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• Younger patients, good prognosis• Contiguous, predictable spread• Reed-Sternberg cell• Several subtypes
Things you must know
Hodgkin Lymphoma
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Reed-Sternberg cell
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Hodgkin lymphoma
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• Malignant proliferation of plasma cells• Monoclonal gammopathy• Decreased normal immunoglobulins• Osteolytic lesions
Multiple Myeloma
Things you must know
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• Weakness• Infections• Renal failure• Bone pain• Hypercalcemia
Clinical Features of Multiple Myeloma
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Bone marrow: tons of plasma cells
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Blood: rouleaux
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Osteolytic lesions
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Normal serum protein electrophoresis
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Serum protein electrophoresis in myeloma
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• Chemo and radiation• Bone marrow transplant• 5 year survival with chemo only: 20%
Treatment of Multiple Myeloma
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