Title: MLAB 1315 Hematology Keri BrophyMartinez
1MLAB 1315- HematologyKeri Brophy-Martinez
2Chronic Lymphocytic Leukemia
- This is a general category of conditions known as
lymphoproliferative disorders. - General requirements for diagnosis
- Peripheral blood (gt5000 ALC) and bone marrow
lymphocytosis (gt30) - Lymphocytes are small to slightly larger than
normal with mature appearance - Nucleus has a condensed pattern
- Smudge cells (bare nuclei) are common. (To
eliminate or reduce smudge cells, mix 4 drops
blood with 1 drop albumin before making smear.) - It is most frequently a leukemia involving B
cells
3Chronic Lymphocytic Leukemia
- Clinical features
- Occurs in persons gt50 old
- Men are affected more than women 21
- Chronic fatigue, infection are result of bone
marrow replacement of normal cells with
lymphocytes. - Skin and organ infiltration
- Median survival is 4-5 years, with 30 of
patients surviving 10 years
4Chronic Lymphocytic Leukemia
- Treatment
- Usually treatment is not required until
lymphocytosis causes other cells to be crowded
out resulting in infections. - Treatment depends on the stage at which the
disease is diagnosed and is usually for the
symptoms, not the disease. - Radiation of localized infiltration
- Chemotherapy given according to stage of disease
- IV gamma globulin for prevention of infection
- Bone marrow transplant done on aggressive cases
- Staging of CLL is done using the Rai system
5Other lymphoproliferative disorders
- Prolymphocytic leukemia
- gt55 of circulating prolymphocytes
- Hairy cell leukemia (HCL)
- Lymphocytes have fine, hairlike, irregular
cytoplasmic projections - Cytochemical stain is strong acid phosphatase
reaction that is not inhibited by tartaric acid
(TRAP) - Bone marrow aspirate may be difficult to obtain
due to marrow fibrosis. Bone marrow biopsy shows
hairy cells separated from each other by a clear
zone with a fried egg appearance. - Hairy cell leukemia can be treated with a
one-time chemotherapy regimen with a good
prognosis of long-term survival.
6Other lymphoproliferative disorders cont
- Sezary syndrome
- Leukemic phase of the most common cutaneous
T-cell lymphoma, mycosis fungoides. - Sezary cell is the size of a small lymphocyte and
has a dark staining, hyperchromic nuclear
chromatin pattern with numerous fold and grooves
referred to as cerebriform. - Diagnosis is confirmed by skin biopsy and
presence of Sézary cells.
7Other lymphoproliferative disorders cont
- Plasma cell dyscrasias - to be discussed later.
8Chronic myelogenous leukemia (CML)
- Aka Chronic granulocytic leukemia (CGL)
- This is a clonal myeloproliferative disorder of
the hematopoietic pluripotent stem cell that has
undergone neoplastic transformation. It is
characterized by an excessive increase of
granulocytes and their precursors in the
peripheral blood.
9Chronic myelogenous leukemia (CML)
- General characteristics
- 90-95 of patients have the Philadelphia
chromosome - t(922) - Marked leukocytosis with all stages of
granulocytic cells - Organomegaly especially the spleen
- Primarily seen in people 30-50 years of age
- Etiology is unknown
10Chronic myelogenous leukemia (CML)
- Phases of the disease
- Chronic
- Controllable with chemotherapy
- Lasts 2-5 years
- Chemotherapy-resistant (accelerated)
- Lasts 6-18 months
- Blastic acute
- Patient undergoes blast crisis
- Generally unresponsive to treatment
11Chronic myelogenous leukemia (CML)
- Clinical features
- Weakness
- Night sweats
- Weight loss
- Bone tenderness
- Abdomen fullness
12Chronic myelogenous leukemia (CML)
- Laboratory features
- Extreme leukocytosis (WBC gt 100,000 x 109)
- Marked left shift
- Normochromic-normocytic anemia (Hgb lt 10 g/dL)
- NRBCs
- Bone marrow ME ratio is 101
- Low LAP score (leukemoid reaction has high LAP)
13Chronic myelogenous leukemia (CML)
- Treatment
- Chemotherapy to reduce the myeloid mass
- Bone marrow transplant
- Interferon