The association of hemophilia A (AHA) and myelofibrosis, a bone marrow disorder, is uncommon and rarely described but in a recent case study, researchers at the Centre Hospitalier Metropole Savoie, in France, reported a patient with AHA secondary to hematological malignancy whose rapid and challenging diagnosis — and timely treatment — was essential to the patient’s survival.
“Myelofibrosis and acquired hemophilia A: a case report,” published in the Journal of Medical Case Reports, is only the second in published literature to provide valuable findings for the clinical handling of the disease.
AHA is a bleeding disorder that results from the development of antibodies against factor VIII (FVIII) treatment and is associated with autoimmune disorders, malignancies, pregnancy, dermatological disorders, and drug reactions. Surgery is suggested as a possible cause of the disorder. The first sign of AHA is usually a massive episode of life-threatening bleeding in a patient without history of coagulation disorders.
Myelofibrosis (MF) is a bone marrow disorder that impairs the bone marrow’s ability to produce blood cells, which leads to severe anemia, weakness, and sometimes to enlarged spleen and liver. According to the case study authors, only one case of MF and AHA had been previously reported. In the new case, they reported a 66-year old man who had been diagnosed with MF four years prior to the case study.
At diagnosis, the patient’s MF-related anemia was first treated with recombinant human erythropoietin. Because the drug was ineffective, it was stopped after 3 months and the patient then received regular red blood cell transfusions. With the intention of halting the anemia, the patient’s spleen was removed.
After the intervention, the patient experienced hemorrhagic shock due to bleeding and needed a second intervention. Finally, a deficiency in FVIII coagulant activity was detected through coagulation tests — FVIII inhibitors showed positive, and the diagnosis of AHA was made.
Clinical remission of the otherwise fatal condition was achieved through combination of hemostatic (causing bleeding to stop) therapy, immunosuppressive treatment (steroids and rituximab), and anti-leukemia therapy. For researchers, the intervention highlighted the importance of treating underlying disease in AHA cases.
The authors concluded: “The findings in this case indicate that rapid and accurate diagnosis, effective hemostatic therapy, and timely treatment for underlying disease are important in the management of AHA.”
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