Is myelofibrosis treatable?
Q: My father is 60 years old and has diabetes type 2 since 12 years. He is suffering from myelofibrosis. His TLC is 39,700 and Hb is 12.5. He is taking Hydroxy urea 500 4/7. Can he undergo a bone marrow transplant? If yes, where?
A:Myelofibrosis, or agnogenic myeloid metaplasia, belongs to a group of disorders called myeloproliferative diseases (MPDs) that are all characterised by an increase in one of the blood cell lines (red cells, white blood cells or platelets) with overlap of findings that may at times make exact categorisation difficult. The condition may transform into another MPD or evolve into acute leukaemia. The disease is difficult to treat and the treatment is mainly supportive involving transfusion in case of severe anaemia or thrombocytopaenia or chemotherapy. Chemotherapy acts as a palliative and is given to reduce the cell counts and control symptoms caused by the enlarged spleen & liver. Drugs include hydroxyurea, interferon, cladribine, etc. No curative treatment is available though there are reports that allogeneic bone marrow transplantation (BMT) may be effective in some cases leading to long-lasting, complete remissions with regression of marrow fibrosis. Elderly patients with severe anaemia, chromosomal abnormalities or osteosclerosis have a poor outcome despite BMT. Even with an HLA-identical sibling, the mortality rate at the end of 1 year is about 30%. You can contact the Christian Medical College, Vellore, Tata Memorial Hospital, Mumbai, Raheja Hospital, Mumbai or I.R.C.H. at A.I.I.M.S., Delhi.