1. Chronic leukemia
Myeloid- CML
Lymphoid- CLL
Hairy cell leukemia

2. CML
 A clonal stem-cell disorder,
categorised as a MPD
 Characterised by Philadelphia chromosome-
t(9:22)(q34:q11)
 BCR gene from chr. 22 fuses with ABL gene
on chr. 9, producing a tyrosine kinase of
210 kDa, that speeds cell division
 Predominantly myeloid proliferation

3. Diagnosis
 Afflicts middle-aged population
 Clinical presentation-
 Incidentally detected raised neutrophil count,
with left shift
 Splenomegaly causing LUQ heaviness
 Anemia & thrombocytopenia
 Compatible bone marrow examination
 Philadelphia chromosome detected by
cytogenetics

4. Phases of CML
 Chronic phase-
 Raised TLC with left shift, splenomegaly, anemia
 Accelerated phase-
 Worsening TLC/s’megaly, unresponsive to treatment
 10-19% myeloblasts in BM
 >20% basophils in blood/BM
 Platelets- <1 lakh/>10 lakh
 Blast crisis-
 >20% myelo/lymphoblasts in BM, like acute leukemia

5. Treatment
 Chronic phase-
 Tyrosine kinase inhibitors- Imatinib,
Nilotinib, Dasatinib
 Allogeneic SCT- only curative Rx
 Hydroxyurea
 Interferon alpha 2b ± cytarabine
 Blast crisis- treated like AML/ALL,
with poor results

6. CLL
 Most common leukemia,
classified as a type of NHL by WHO
 Affects B lymphocytes
 Incidence increases with age
 Mostly detected incidentally,
based on lymphocytosis on CBC
 AIHA & frequent infections are seen
 Flow cytometry confirms clonality,
based on presence of CD19/20/5 & CD23

7. Staging
 Rai-
 Low- lymphocytosis only
 Intermediate- with LNE ± HSmegaly
 High- with anemia/thrombocytopenia
 Binet-
 A- Hb>10, platelet>100000, <3 LN areas involved
 B- Hb>10, platelet>100000, >3 LN areas involved
 C- Hb<10 ± platelets<100000

8. Prognosis
 Stage- higher is worse
 β2 microglobulin- higher is worse
 CD38 or ZAP-70 markers for high-risk
 del 17p/11q- worse
 del 13q- good
 Richter syndrome- transformation to
high-grade lymphoma- bad

9. Treatment
 CLL progresses slowly
 All need not be treated
 Disfiguring/obstructive LNE, anemia &
thrombocytopenia indicate need for treatment
 Drugs- not curative in majority
 Chlorambucil- oral
 Fludarabine + Cyclophosphamide/Rituximab
 COP/CHOP
 Alemtuzumab- CD52 Mab
 AIHA- steroids
 Frequent infections- prophylactic IVIG

10. Hairy cell leukemia
 Rare, mostly males >50 years of age
 A mature B-cell neoplasm
 Causes massive splenomegaly &
pancytopenia
 Dx-
 Pancytopenia with splenomegaly
 Hairy-cells (abnormal WBC) in blood/ bone marrow
 BM with TRAP +ve cells
 Flow cytometry- CD103,22,11c +ve
 Increased TNF-α & IL-2R

11. Treatment
 Not all need treatment
 Severe neutropenia or
thrombocytopenia needs to be treated
 Purine analogs- Cladribine or
Pentostatin
 2nd
line- Rituximab or IFN-α
 Splenectomy- when troublesome