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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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