Chronic Leukemia, Lymphoma, and Myeloma · 2019-10-23 · 1 Kristine Krafts, M.D. Chronic Leukemia, Lymphoma, and Myeloma 1 Leukemias • Acute leukemias • Chronic leukemias Lymphomas

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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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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


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All cases of CML have the Philadelphia chromosome!

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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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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


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• Most common leukemia in adults

• Small, mature B lymphocytes

• Weird immunophenotype: CD5+

• Decreased normal immunoglobulins

Chronic Lymphocytic Leukemia


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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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Lymphomas• Hodgkin lymphoma• Non-Hodgkin lymphoma


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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


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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


MALT Lymphoma

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• Skin lesions• Blood involvement• Cerebriform lymphocytes• T-cell immunophenotype


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


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


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


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


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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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Chronic Leukemia, Lymphoma, and Myeloma · 2019-10-23 · 1 Kristine Krafts, M.D. Chronic Leukemia, Lymphoma, and Myeloma 1 Leukemias • Acute leukemias • Chronic leukemias Lymphomas