White Blood Cell Leukemias, Lymphomas, and Multiple Myeloma

Comprehensive vocabulary flashcards covering the pathophysiology, clinical features, and classification of Leukemias, Lymphomas, and Multiple Myeloma based on the lecture by Mark A. Fischione, M.D.

What is leukemia?

malignant disease involving WBC precursors in the bone marrow and increased malignant WBCs in the peripheral blood

What are the classes of leukemias?

myeloid (granulocytic monocytic)

lymphoid (lymphocytic)

What is HTLV1?

T cell lymphotropic retrovirus

may cause leukemia or lymphoma

What is EBV?

herpesvirus that infections B lymphocytes

may cause Burkitt lymphoma

How does leukemia differ in adults and children?

children are usually acute

adults are usually chronic

How does bone marrow appear in leukemia?

infiltrated with malignant cells

How does a peripheral smear appear in leukemia?

increased number of immature blood cells

What are complications of leukemia?

anemia

recurrent infections

uncontrolled bleeding

How does leukemia present clinically?

anoxia, bleeding, overwhelming infection

What is acute lymphoblastic lymphoma (ALL)?

immature b cell lymphoma that has lymph node involvement

most common leukemia in children

What patient demographics are seen in acute lymphoblastic lymphoma (ALL)?

children younger than 5 and elderly

What are characteristics of acute lymphoblastic lymphoma (ALL)?

massive infiltration of bone marrow with immature lymphoid cells that spill into peripheral blood

What is the clinical presentation of acute lymphoblastic lymphoma (ALL)?

recurrent infection, generalized weakness, bleeding into the skin

bone pain and arthralgia

enlarged lymph nodes with mild splenomegaly

What is the prognosis of ALL?

remission in most patients with chemotherapy, potential cure

fatal is untreated

What patient demographics are associated with acute myeloid leukemia (AML)?

older individuals

What is the most common acute leukemia?

acute myeloid leukemia (AML)

What are characteristics of acute myeloid leukemia (AML)?

massive infiltration of the bone with immature malignant myeloblasts that spill into peripheral blood

What is the myoblast requirement for a diagnosis of acute myeloid leukemia (AML)?

more than 20% of myeloblasts in the bone marrow

What risk factors are associated with acute myeloid leukemia (AML)?

radiation

cytotoxic chemotherapy

benzene exposure

smoking

What are the 4 types of acute myeloid leukemia (AML)?

AML with recurrent genetic abnormality

AML with multilineage dysplasia

AML therapy related chemo or benzene related

AML not otherwise classified

What are auer rods?

cytoplasmic inclusions (stick shaped) specific to acute myeloid leukemia (AML)

How is acute myeloid leukemia (AML) diagnosed?

immunophenotypic and cytogenetic studies

myeloperoxidase and nonspecific esterase markers to label myeloid cells

What is the clinical presentation of acute myeloid leukemia (AML)?

pancytopenia with infections (fungal)

anemia and cutaneous bleeding

bone pain

What physical exam findings are seen in acute myeloid leukemia (AML)?

splenomegaly, lymphadenopathy, and testicle enlargement

possible retinal infiltrates

What is the prognosis for acute myeloid leukemia (AML)?

death within 6 months if on treatment

remission in 50% with chemo, but majority relapse

longer survival with bone marrow transplant

What patient demographics are associated with chronic lymphocytic leukemia (CLL)?

increasing age

What are characteristics of the cells in chronic lymphocytic leukemia (CLL)?

indistinguishable from normal mature lymphocytes

suspect if numbers in peripheral blood are more than 15k/microL

What type of lymphoma shares features with chronic lymphocytic leukemia (CLL)?

small cell lymphocytic lymphoma

What is the prognosis for chronic lymphocytic leukemia (CLL)?

survival 7-9y from diagnosis

What is the definition of chronic myeloproliferative disorder?

clonal hematogenous stem cell disorders with increased proliferation of one or more myeloid lineages

What patient demographics are associated with chronic myeloproliferative disorder?

adults 40-80

What are the types of chronic myeloproliferative disorders?

chronic myelogenous leukemia (CML)

polycythemia vera

idiopathic myelofibrosis

essential thrombocytopenia

What is the most common chronic myeloproliferative disorder?

chronic myelogenous leukemia (CML)

What is the definition of chronic myelogenous leukemia (CML)?

malignant disease of myeloid stem cell precursors with granulocytic differentiation

What patient demographics are associated with chronic myelogenous leukemia (CML)?

adults of increasing age

What environmental risk factors are associated with chronic myelogenous leukemia (CML)?

radiation and benzene

How does the bone marrow appear in chronic myelogenous leukemia (CML)?

overgrown with malignant stem cells and descendants, may result in high WBC count in peripheral blood

How does the Philadelphia Chromosome related to chronic myelogenous leukemia (CML)?

95% of patients with CML have the chromosome

5% without it have a worse prognosis

What causes a Philadelphia Chromosome?

reciprocal translocation between chromosomes 9 and 22 shown by cytogenetic and FISH studies

What is a Philadelphia Chromosome?

shortened chromosome 22 with 2 genes not normally together that encode uncontrolled tyrosine kinase, leading to cancer

What causes tyrosine kinase formation of the Philadelphia Chromosome?

ABL gene encoding tyrosine kinase fuses with BCR gene and gets activated

What is the clinical progression of chronic myelogenous leukemia (CML)?

slow onset of anemia, fatigue, and increased infection

progression to splenomegaly and thrombosis from clotting

progresses to blast crisis

What is a blast crisis?

progression of CML into an accelerated phase that resembles acute leukemia

usually causes death

What is the prognosis of chronic myelogenous leukemia (CML)?

poor response to chemo, increased survival with bone marrow transplant

death within 3-5 years

Non-Hodgkin’s Lymphoma (NHL)

A category of lymphomas separated into T-Cell and B-Cell types derived from immature or mature cells.

Follicular Lymphomas

Low-grade B-Cell lymphomas where the follicular structure is preserved and tumor cells resemble mature lymphocytes; the second most common subtype ($20\%$).

Diffuse Large Cell Lymphoma

The most common B-Cell Lymphomas ($40\%$) characterized by complete effacement of normal lymph node architecture and infiltration of large lymphoblasts.

Burkitt’s Lymphoma

A high-grade malignant tumor associated with an $8:14$ translocation and EBV infection, often presenting with extranodal involvement of the mandible.

Reed-Sternberg Cell

A diagnostic cell for Hodgkin’s Disease featuring a bilobed, mirror-image nucleus with prominent nucleoli reminiscent of an "owl’s eye."

Ann Arbor Stage I

Hodgkin’s Disease involvement of a single lymph node region.

Ann Arbor Stage II

Hodgkin’s Disease involvement of two or more lymph node regions on the same side of the diaphragm.

Ann Arbor Stage III

Involvement of lymph node regions on both sides of the diaphragm, which may include the spleen.

Ann Arbor Stage IV

Multiple or disseminated foci of involvement of one or more extralymphatic organs.

Multiple Myeloma

A plasma cell neoplasm characterized by monoclonal proliferation, skeletal destruction at multiple sites, and the secretion of immunoglobulins.

Monoclonal Spike

A sharp peak detected on serum protein electrophoresis representing the uniform immunoglobulins secreted by malignant plasma cells in Myeloma.

Bence-Jones Proteins

Light chains of immunoglobulins secreted in the urine which can damage renal tubules and contribute to renal failure.

Rouleax Formation

The stacking of RBCs in peripheral blood due to an increased sedimentation rate, commonly seen in Multiple Myeloma.