CHRONIC MYELOID LEUKEMIA

Leukemias are malignant Neoplasms of the haematopoetic stem cells characterized by diffused replacement of bone marrow by Neoplastic cells with or without involvement of the peripheral blood.

➡️ Chronic leukaemia is a disease of insidious onset, affecting middle aged & young adults (CML)

🔅CLINICAL FEATURES:

  1. Gradually developing Anaemia
  2. Hepatosplenomegaly (In CML, a massive splenomegaly reaching upto the umblicus) Therefore, acute pain due to splenic infarction
  3. Bleeding tendencies
  4. Hypermetabolism
  5. Bone pain
  6. Juvenile CML: Lymph node enlargement, frequent infections, haemorrhagic manifestations & facial rash

🔅LAB. FINDINGS:

  • Thick and sticky & shows a wide buffy coat in the haematocrit tube
  • Anaemia – Moderate, normocytic normochromic
🔘 1 lakh - 5 lakh/cu.mm

🔘 Differential count reveals more mature forms in Peripheral smear, complete spectrum of granulocytes, myelocytes & band cells (50%)

🔘 Blast cell > 10% of leukocytes

🔘 Phases:

1. Chronic phase:

➡️ Myeloproliferative disorder – excessive proliferation of myeloid cells & mature segmented Neutrophils

➡️ Basophils upto 10% (feature of CML)

2. Accelerated phase:

  • ⬆️ Anaemia
  • Blast count: 10-20%
  • Basophils: 20%
  • Platelet: ⬇️ 1 lakh/µl
  • Leucocytosis

3. Blastic phase: Myeloid blast crisis in CML resembles AML >20%

  1. Cellularity: Hypercellular fat spaces replaced by Myeloid cells.
  2. Myeloid cells: ⬆️ M:E ratio
  3. Erythropoiesis: Normoblastic
  4. Megakaryocytes: Conspicuous, smaller in size
  5. Chromosomal abnormalities: The philadelphia chromosome is present in about 90% of the cases of CML

• neutrophil alkaline phosphatase (NAP) activity is markedly reduced in CML

🔅TREATMENT:

  • The standard treatment for chronic phase CML is a tyrosine kinase inhibitor (TKI) like imatinib oral therapy. Imatinib works by reducing the production of abnormal white blood cells.
  • Allogenic bone marrow transplants.
  • Interferon was once the best treatment for CML, but now, the tyrosine kinase inhibitors are the mainstay of treatment and interferon is rarely used. To treat CML, this drug is most often given as a daily injection (shot) under the skin. It may also be injected into a muscle or vein. It’s given for many years.
  • Chemotherapy: Today, chemo may be used to treat CML when the TKIs have stopped working. It’s also used as part of a stem cell transplant.
  • Others:
  • Splenectomy
  • Splenic irradiation
  • Leukapheresis

🔘 Splenectomy and splenic irradiation have been used in patients with large and painful spleens, usually in the late phase of CML. This is rarely needed in patients whose disease is well controlled.

🔘 For those patients unable to tolerate chemotherapy, leukapheresis is a viable option.

Dr. Mehnaz Memon🖊


References:

  1. Textbook of Pathology, Harsh Mohan; Internet articles
  2. Image source: Google

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