Icm Ii Hematology-Oncology: Lymphoid Disorders

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Presentation
- Bone marrow failure
• Anemia – fatigue, weakness, shortness of breath
• Thrombocytopenia – increased bruising and bleeding
• Leukopenia/neutrapenia – infections
- Enlarged lymph nodes and/or splenomegaly
- Mediastinal mass
- Neurologic complaints (headaches, double vision, swallowing difficulty, etc) from leukemia in the central nervous system

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Acute Lymphocytic Leukemia(ALL)

Epidemiology
– Incidence 1-3/100,000/year
– 3,000 new cases of ALL/year
– Peak incidence at 2-3 years
– Second peak after 80 years
– 10-20% of adult leukemias, but 90% of childhood leukemias
Pathology
- Acute Leukemia is by definition a cancer of bone marrow progenitors (progenitors are immature marrow cells still capable of division)
- Lymphoblasts (immature lymphocytes) proliferate and are unable to mature into normal cells
- These blasts eventually dominate the bone marrow, suppressing normal hematopoiesis – therefore decreases red cell, platelet, and neutrophil production
- By definition acute leukemia must have 20% leukemic blasts in the bone marrow
- Can be T or B cell origin
- Often associated with chromosomal (cytogenetic) abnormalities, including the Philadelphia chromosome (9;22 translocation). This particular translocation is associated with a very poor response to conventional treatments

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- Often found incidentally on routine blood work (patient asymptomatic)
- Can present secondary to bone marrow failure
• Anemia – fatigue, weakness, shortness of breath
• Thrombocytopenia – increased bruising and bleeding
• Neutropenia (lack of neutrophils) – infections
- Lymphadenopathy/Splenomegaly
- B symptoms (night sweats, weight loss, fever).

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Chronic Lymphocytic Leukemia(CLL)

Epidemiology
- Disease of advancing age
- Approximately 10,000 new cases per year
Pathology
- Proliferation of mature lymphocytes in the bone marrow
- Majority are of B-cell origin
- Usually an indolent process
- Does not convert to ALL

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Presentation
- Lymphadenopathy
- B symptoms (night sweats, weight loss, fever). These represent a worse prognosis, and are added to the staging
- Can present in extra-nodal sites

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Non-Hodgkin’s Lymphoma(NHL)

Epidemiology
- NHL is rapidly increasing in incidence in the U.S., with 50,000 new cases/yr and 20,000 deaths/yr.
- Age and any immunodeficiency (especially AIDS) increase risk.
Pathology
- Malignancy can occur at any stage of lymphocyte development in the lymph node
- There are multiple histologic types with differing proliferation rates
o Indolent (low-grade)– this grows slowly but is incurable (average survival 8 yrs) – an example is Follicular lymphoma
o Aggressive (intermediate) grade – this grows more quickly, but is curable- an example is Diffuse large cell lymphoma- the most common type of NHL, which has a 50-60% 5-year survival
o Leukemic equivalent (high-grade) –these grow extremely rapidly and are potentially curable - an example is Burkitt’s lymphoma.
- NHL usually starts in the lymph nodes (or other lymphoid tissue) and spreads both via the lymphatics and hematogenously to adjacent nodes, the spleen, liver and/or bone marrow
- Stage I- single node region, Stage II- 2 or more nodal groups on the same side of the diaphragm, Stage III- nodes on both sides of the diaphragm involved, Stage IV- any extra-lymphatic involvement (e.g. liver, marrow).

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Hodgkin’s Lymphoma or Disease (HD)

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Hodgkin’s Lymphoma or Disease (HD)

Epidemiology
- HD is uncommon, 8,000 new cases/yr with 2,000 deaths/yr
- Incidence demonstrates a bimodal distribution – peak ages 15-35 and second smaller peak after age 50
Pathology
- The characteristic malignant cell is the CD30+ Reed-Sternberg cell, thought to be an antigen presenting cell in the lymph node
- HD starts in the nodes and spreads predominantly via the lymphatics to adjacent nodes.
- Stage I- single node region, Stage II- 2 or more nodal groups on the same side of the diaphragm, Stage III- nodes on both sides of the diaphragm involved, Stage IV- any extra-lymphatic involvement (e.g. liver, marrow)
Presentation
- Lymphadenopathy
- B symptoms (night sweats, weight loss, fever). These represent a worse prognosis, and are added to the staging

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Presentation
- Bone pain from lytic lesions or fractures
- Weakness and light-headedness from anemia
- Infection from hypogammaglobulinemia
- Nausea and vomiting, edema from renal failure
- Confusion from hypercalcemia

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Plasma Cell Dyscrasias

Multiple Myeloma – most common type of plasma cell dyscrasia

Epidemiology
- Uncommon, 13,000 new cases/yr, causing 9,000 deaths/yr.
- Advancing age, males, African-American race, and radiation exposure associated with an increased risk.
Pathology
- A malignancy of antibody-producing B-cells. They typically produce a monoclonal antibody that can suppress other antibody formation (hypogammaglobulinemia)
- Antibodies are typically IgG and IgA (not IgM)
- Myeloma can create lesions in cortical bone leading to hypercalcemia, fractures, and spinal cord compression
- Antibody deposition, hypercalcemia, hyperuricemia all can lead renal insufficiency
- Myeloma cells typically stay in the marrow but may also produce malignant masses, called plasmactyomas that can be found anywhere in the body
- Not a curable disorder

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Waldenstrom’s Macroglobulinemia

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Waldenstrom’s Macroglobulinemia –
- Plasma cell dyscrasia associated with increased production of IgM
- Does not cause bone destruction or renal failure
- Increased amount of IgM can cause hyperviscosity – slow movement of blood that can lead to decreased CNS function from decrease oxygen delivery

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Rouleaux Formation

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Rouleaux Formation: In a blood smear, RBC stack up on top of each even in the Good goldilocks area. This is a sign that serum protein levels are high. When does this occur? In plasmacytomas or in multiple myeloma.