Chronic Lymphoproliferative Disorders - Krafts Flashcards
What are the four types of Chronic Lymphoproliferative Disorders
- Chronic Lymphocytic Leukemia
- Hairy Cell Leukemia
- Prolymphocytic Leukemia
- Large Granulated Lymphocyte Leukemia
What are characteristics of ALL Chronic Lymphoproliferative Disorders?
- Malignant proliferation of lymphocytes in blood and bone marrow
- Many disorders, CLL most important
- Occur only in adults
- Long course; indolent but incurable
- appear slowly over time without noticing
- not dividing rapidly, so hard to kill with drugs
- not possible to do bone marrow transplants (predominantly in lymph nodes)
What are the clinical findings in CLL?
- Older (>40 yoa)
- Asymptomatic at first
- Later, organ infiltration
What is the blood/bone marrow morphology in CLL?
- BLOOD:
- proliferation of mature-appearing lymphocytes (small cells, with clumped/smudged chromatin)
- monomorphic (look alike, cut/paste)
- white count may be massively elevated, or just slightly above normal
- BONE MARROW:
- Proliferation of mature lymphocytes.
- Focal involvement (hypercellular, no fat)
- The more marrow involved, the worse the prognosis.
What is the immunophenotype of CLL?
- Positive for:
- B-cell antigens
- T-cell antigen = CD5
- Negative for:
- TdT (lymphoblasts)
What are the laboratory findings in CLL?
- Abnormal CBC
- mildly-severely increased WBCs
- Hypogammaglobulinemia
- normal immunoglobulins decreased
- Flow cytometry
- markers for CD5 and CD20 (CLL)
What is the pathophysiology of CLL (genetic abnormality)?
- bcl-2 gene rearrangement
- makes gene more powerful
- expressed all the time (immortal)
- normal bcl-2 prevents apoptosis
What is the treatment of CLL?
- Conservative
- don’t always treat because patients are older
- not rapidly growing disease
- treatment often worse than the disease
- Reduce organomegaly
- Treat infections
- vaccinate
What is the prognosis for patients with CLL?
- “Counts”
- normal hemoglobin count
- platelet count
- Adenopathy
- big lymph nodes = worse prognosis
- Bone marrow pattern
- Chromosomal abnormalities
- Mean survival = 9 years
- Death usually from infection
What are the clinical findings in Hairy Cell Leukemia?
- Big spleen (splenomegaly)
- NO lymphadenopathy
- Older (>40)
- M:F = 5:1
What is the blood/bone marrow morphology in Hairy Cell Leukemia?
- Blood:
- pancytopenia
- few leukemic cells that are present have a shaggy appearance – the cytoplasm appears to have kind of a messy, blurry border
- monocytopenia is always present
- Bone marrow:
- hypercellular
- loosely structured (“moth-eaten” appearance)
- fibrotic response (reticulin fibrosis)
- like “chicken wire”
What are the special lab studies that can be performed in HCL?
- Cytochemistry
- TRAP+ stain (specific for hairy cells)
- Immunophenotype
- Positive for B-cell antigens, CD25, CD11c
- Negative for CD5
What are the typical laboratory findings in HCL?
- Pancytopenia (usually)
- Monocytopenia (always)
What is the treatment for HCL?
Chemotherapy
(often just one shot)
What is the prognosis of a patient with HCL?
GOOD!
(only need one shot of chemo)
