Myelodysplastic syndromes (MDS)

Myelodysplastic syndromes (MDS)


MYELODYSPLASTIC SYNDROME (MDS)

  • MDS is a heterogeneous group of disorders characterised by both, cytopenia, due to bone marrow failure & development of AML.
  • Also called as preleukemic syndromes or dysmyelopoietic syndromes.
  • More common in males.
  • Seen mainly disease of elderly (mean age 68 years).

Etiology-

  • Radiation
  • Chromosomal aberration.

Pathogenesis-

  • Common karyotypic abnormalities includes-
  • Monosomy 5 or 7
  • Deletions of 5q, 7q & 20q
  • Trisomy 8
  • All types of MDS transforms to AML but frequently seen with t- MDS.

Morphology-

a) Erythroid lineage

  • Ringed sideroblasts  is an erythroblasts with iron- laden mitochondria.
  • Nuclear budding abnormalities

b) Granulocytic lineage-

  • Dohle bodies (damaged RER)
  • Pseudo- Pelger- Huet cells

c) Megakaryocytic lineage-

  • Pawn ball megakaryocytes with single nuclear lobe.

Clinical features-

  • Anaemia, fatigue, weakness
  • Fever, rashes
  • Weight loss
  • Splenomegaly
  • Sweet syndrome

Investigations-

I) Blood findings-

  • Anaemia- macrocytic or dimorphic
  • Thrombocytopenia
  • Neutrophils are hyposegmented & hypogranulated (pantocytopenia)

II) Bone marrow findings-

  • Erythroid series- dyserythropoiesis appear
  • Ring sideroblasts
  • Megakaryocytes decreased

Treatment-

  • Azacitidine & decitabine used in BM failure.

Exam Important

  • MDS is a heterogeneous group of disorders characterised by both, cytopenia, due to bone marrow failure & development of AML.
  • Common karyotypic abnormalities includes-
  • Monosomy 5 or 7
  • Deletions of 5q, 7q & 20q
  • Trisomy 8
  • All types of MDS transforms to AML but frequently seen with t- MDS.

Morphology-

a) Erythroid lineage

  • Ringed sideroblasts  is an erythroblasts with iron- laden mitochondria.

b) Granulocytic lineage-

  • Dohle bodies (damaged RER)
  • Pseudo- Pelger- Huet cells

c) Megakaryocytic lineage-

  • Pawn ball megakaryocytes with single nuclear lobe.
  • Neutrophils are hyposegmented & hypogranulated (pantocytopenia)

Bone marrow findings-

  • Erythroid series- dyserythropoiesis appear
  • Ring sideroblasts
Don’t Forget to Solve all the previous Year Question asked on Myelodysplastic syndromes (MDS)

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