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Acquired Thalassemia in Myeloproliferative or Myelodysplastic Disorders

Description

A patient with a myelodysplastic or myeloproliferative disorder may develop an acquired thalassemia during the course of the disease. This is related to a defect in hemoglobin synthesis secondary to a deletion of a gene or inactivating somatic mutation.


 

Synonym: alpha Thalassemia associated with myeloid malignancy, ATMDS syndrome

 

Features:

(1) absence of thalassemia prior to development of the hematologic disorder

(2) presence of alpha or beta thalassemia after onset of the hematologic disorder

 

Alpha-thalassemia is the more common form of acquired thalassemia.

 

The patient may develop:

(1) hemoglobin H (with 4 beta chains following loss of alpha chain production) with positive supravital staining using brilliant cresyl blue

(2) a microcytic anemia unrelated to iron deficiency

 


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