Chronic Leukemia

1. CHRONIC MYELOGENOUS LEUKEMIA
2. CHRONIC LYMPHOCYTIC LEUKEMIA
3. HAIRY CELL LEUKEMIA

CHRONIC MYLOID LEUKEMIA (CML)

CML is a myeloproliferative disorder characterized by over production of myeloid cells.

• CYTOGENETICS – PHILADELPHIA CHROMOSOME
• ONCOGENE – bcr/abl
• PHILADELPHIA POSITIVE – 95%
• PHILADELPHIA NEGATIVE – 5%

CLINICAL BEHAVIOR

• CHRONIC PHASE
• ACCELERATED PHASE
• BLAST CRISIS

DIFFERENTIAL DIAGNOSIS

• REACTIVE LEUCOCYTOSIS
• OTHER MYELOPROLIFERATIVE DISORDERS

MANAGEMENT

CURATIVE:

ALLOGENEIC – BMT.

1.MYELOABLATIVE – CHEMO+GVL.

2.NON MYELOABLATIVE – CHEMO+GVL.

3.DLI – GVL.

AUTOLOGOUS – BMT.

PALLIATIVE:

• IMATINIB MESYLATE – 400mg/d – p.o.
• INTERFERON ALPHA + CYTARABINE.
• HYDROXYUREA 40mg/kg/d – p.o
• BUSULPHAN 0.1 mg/kg/d – p.o.

CHRONIC LYMPHOCYTIC LEUKAEMIA (CLL)

PROGRESSIVE ACCUMULATION OF  LYMPHOCYTES IN BLOOD BONEMARROW & LYMPHATIC TISSUE.

GOOD PROGNOSIS – DIFFERENTIATED MATURE B-CELLS WITH SOMATICALLY MUTATED HEAVY CHAIN GENE. DO NOT EXPRESS ZETA ASSOCIATED PROTEIN ZAP70.

POOR PROGNOSIS – LESS DIFFERENTIATED B-CELLS  WITH UNMUTATED HEAVY CHAIN IgV GENES AND EXPRESS HIGH LEVEL OF ZAP 70.

DIAGNOSIS

• HIGH WBC COUNT.
• BLAST < 5%.
• PLATELET COUNT NORMAL OR HIGH.
• BONEMARROW – MYELOID HYPER PLAESIA MYELOBLAST < 5%.
• bcr/abl GENE DETECTED BY PCR.
• ACCELERATED PHASE – ANAEMIA THROMBOCYTOPENIA & INCREASED NUMBER OF BLASTS.
• BLAST PHASE – BONEMARROW BLASTS > 20%

STAGING

• RAI STAGING SYSTEM
• BINET STAGING SYSTEM

CLINICAL FEATURES

1.FATIGUE.

2.LYMPHADENOPATHY.

3.HEPATOSPLENOMEGALY..

4.FEVER, NIGHT SWEATS, WEIGHT LOSS.

DIAGNOSIS

1.LYMPHOCYTOSIS.

2.BONEMARROW INFILTRATED BY MATURE LYMPHOCYTES.

3.LYMPH NODE HISTOLOGY SMALL LYMPHOCYTIC LYMPHOMA. 4.IMMUNOPHENOTYPINE MATURE B-LYMPHOCYTES – CD 19, CD

5. HYPOGAMMAGLOBULINEMIA

DIFFERENTIAL DIAGNOSIS

1.VIRAL FEVER.

2.SMALL LYMPHOCYTIC LYMPHOMA.

3.MANTLE CELL LYMPHOMA.

COMPLICATIONS

• RECURRENT INFECTIONS.
• AUTOIMMUNE HAEMOLYTIC ANAEMIA & THROMBOCYTOPENIA.
• BONEMARROW FAILURE.
• RICHTER’S TRANSFORMATION

INDICATIONS FOR TREATMENT

• B SYMPTOMS.
• BONEMARROW FAILURE.
• BULKY DISEASE.
• AUTOIMMUNE HAEMOLYTIC ANAEMIA & THROMBOCYTOPENIA. LYMPHOCYTE DOUBLING TIME < 12 MONTHS.
• LOCAL COMPRESSION.
• RECURRENT INFECTIONS.
• ADVANCED DISEASE.
• COSMETIC REASON.

MANAGEMENT

1.CHLORAMBUCIL.

2.CHLOROMBUCIL + PREDNISONE.

3.FLUDARIBINE.

4.COP, CHOP.

5.FLUDARIBINE + CYCLOPHOSPHAMIDE

6.RITUXIMAB

7.ALLOGENIC TRANSPLANTATION.

8.AUTOLOGOUS TRANSPLANTATION

PROGNOSIS

§ STAGE 0 – I               10 – 15 YEARS

§ STAGE II                     5           YEARS

§STAGE III – IV             2           YEARS

HAIRY CELL LEUKEMIA

• 2% OF ALL LEUKAEMIAS
• MEN – WOMEN RATIO    5:1
• MEDIAN AGE OF PATIENT IS 55 YEARS

PATHOLOGY:

HAIRY CELLS IN PERIPHERAL BLOOD, BONEMARROW, LIVER & SPLEEN.

CLINICAL FEATURES

• FATIGUE
• BLEEDING DIATHESIS
• RECURRENT INFECTIONS
• SPLENOMEGALY

LABOROTORY STUDIES

• BLOOD CP – PANCYTOPENIA
• HAIRY CELLS
• BONEMARROW ( HAIRY CELL)
• TRAP – POSITIVE , FIBROSIS
• IMMUNE FLOWCYTOMETRY CD19, CD20, CD22, CD11c, CD25, CD103. SPLENIC MORPHOLOGY – INFILTATION OF RED PULP WITH HAIRY CELLS

DIFFERENTIAL DIAGNOSIS

• MYELOFIBROSIS
• SPLENIC LYMPHOMA
• CLL

MANAGEMENT

INDICATIONS, ANAEMIA, NEUTROPENIA, THROMBOCYTOPENIA

DRUGS

• CLADRIBINE §PENTOSTATIN
• INTERFERON

SPLENECTOMY

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