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



(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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