Incidence of Rarity

Primary Myelofibrosis converted into AML - M7

THE CASE

A 62 year old man experiencing nasal bleeding and weakness visited Nanavati Super Speciality Hospital. His investigations revealed anaemia with leucocytosis and thrombocytosis. His was a known case of primary Myelofibrosis, a disorder in which normal bone marrow tissue is gradually replaced with fibrous tissue. He was prescribed oral Danazole tablet for his condition but in spite of its regular consumption he developed recurrent epistaxis. He had also undergone Splenectomy a year back at a local hospital.

On further investigations his bone marrow aspiration showed 80% blasts. Immunophenotyping revealed positivity for platelet precursor markers ( CD41,CD61). These blasts were negative for other myeloid and lymphoid markers. His diagnosis was positive for Acute Myeloid Leukaemia with megakaryocytic differentiation (FAB AML M7).

THE PROCEDURE

A fraction of patients with Myelofibrosis progress to Acute Myeloid Leukaemia. But Medical and Haemato-Oncologists from Nanavati Hospital diagnosed this rare condition. Considering high risk factors present in this patient and taking into account his age and poor performance status, he was deemed unfit for Allogeneic Stem Cell Transplantation. Thus, Decitabine was started accordingly.

DISCUSSION

Myelofibrosis converting into AML is a very rare phenomenon but our expert Medical and Haemato-Oncologists diagnosed this rare condition. The identification of high risk factors of transformation to AML will allow for early therapeutic intervention and treatment.

Dr. Kshitij Joshi
Associate Consultant-Medical
& Haemato-Oncology
Nanavati Super Speciality
Hospital, Mumbai