ASK DIS: Neutropenia and Thalassemia

patients with thalassemia frequently experience transient episodes of mild or moderate neutropenia irrespective of the chelation therapy they are on

Neutropenia due to Thalassemia

Splenomegaly invariably develops in the symptomatic thalassemias. Splenomegaly can worsen the anemia and occasionally cause neutropenia and thrombocytopenia.
neutropenia occurs significantly more often in patients with thalassemia who have not undergone splenectomy than in those who have
some viral infections, particularly parvovirus, may cause neutropenia independent of chelation therapy
Splenectomy does not causes neutropenia generally. Patients without a functioning spleen have a severe impairment in their ability to cope with specific infections [Pneumococcus, Meningococcus and Haemophilus influenzae]
In some cases (Felty’s Syndrome) splenectomy is actually able to reverse profound neutropenia

Neutropenia due to Iron Chelators

Approximately 6% of patients with thalassemia receiving deferiprone develop neutropenia
Due to the risk of agranulocytosis and associated rare deaths, weekly white blood cell counts are required for all patients receiving this drug.
Some studies showed that not all cases of mild neutropenia during deferiprone therapy develop into agranulocytosis, and suggests that many episodes of neutropenia may not be caused by deferiprone

Management

In deferiprone-treated thalassemia patients who develop agranulocytosis, therapy must always be interrupted
Neutropenia may precede the development of agranulocytosis.
Measure the absolute neutrophil count (ANC) before starting deferiprone therapy and monitor the ANC weekly during therapy.
Interrupt deferiprone therapy if neutropenia develops.
If infection develops, interrupt deferiprone and monitor the ANC more frequently.
Advise patients taking deferiprone to report immediately any symptoms indicative of infection
The present practice is to always discontinue therapy in all patients who experience a decline in neutrophils below 1.5 X 109 /L, irrespective of the potential cause of the neutropenia
For agranulocytosis (ANC < 0.5 x 109 /L), Consider hospitalization and other management as clinically appropriate. Do not resume Ferriprox in patients who have developed agranulocytosis unless potential benefits outweigh potential risks. Do not rechallenge patients

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ASK DIS