Two patients with haemophilia and acute leukaemia

Abstract

Acute leukaemia is the commonest form of malignancy in childhood. The coincidental development of leukaemia in children or adults with haemophilia is extremely rare, although cases of leukaemia and other malignancies have been reported previously in HIV-positive subjects. Of a total of 440 people with haemophilia registered with our society, two were diagnosed with acute leukaemia last year. The development of leukaemia in a subject with haemophilia has previously been reported from our country in 1985, but the negative HIV status of these recent cases is very interesting. The first case involved a 14-year-old boy with moderate haemophilia A, who developed acute lymphoblastic leukaemia (ALL) [French-American-British (FAB) classification L2]. The second subject was a 16-year-old boy who had moderately severe haemophilia A with no previous family history, and developed acute nonlymphocytic (myelomonocytic) leukaemia (FAB-M4). Both patients received conventional chemotherapy and this report discusses the potential problems in management of such cases, including diagnosis and administration of chemotherapy in subjects with a pre-existing haemorrhagic disorder. Extensive cutaneous and mucosal bleeding, as well as bleeds in joints previously affected by haemarthrosis and alterations of haematological values were all initially suggestive of the development of inhibitors against factor VIII, but the appearance of blasts in the peripheral blood and bone marrow led to the definitive diagnosis. The risk of bleeding, due to the combination of both leukaemia and the consequences of the chemotherapy, was overcome by the administration of coagulation factor concentrates (daily initially followed by prophylactic doses after successful induction of remission in both patients). The young patient with ALL is now receiving the maintenance phase of the Children's Cancer Study Group 1961 protocol and is in the 15th month of follow-up, without any complications. The other case relapsed in the seventh month, developing enterobacter sepsis, and died. An important lesson to be learnt from these cases is that the possible diagnosis of leukaemia should not be overlooked in a patient with haemophilia and severe haemorrhagic problems, if the first-line differential diagnosis of inhibitor development against factor VIII (or IX) has been excluded.

The first case report of a patient with simultaneous haemophilia A and accelerated phase chronic myelogenous leukaemia

“…The treatment was 1500 U of FVIII q12 h with the goal of maintaining FVIII activity at 25-50%, as well as cryoprecipitate (3.4 g per dose fibrinogen) and transfusion of platelets. Zulfikar [5] described two patients with haemophilia A, one with ALL and one with AML-M4. The patient with ALL received rhFVIII 20 U kg À1 initially, 30 U kg À1 for bone marrow aspiration, 75 U kg À1 on days 1-4 of chemotherapy, 30 U kg À1 on days 5-21 (induction), 25 U kg À1 every other day during consolidation, 25 U kg À1 every other day during intensification, and on demand during the maintenance phase.…”

A rare case of acute lymphoblastic leukaemia with hemophilia A

“…In literature, 11 cases of hemophilia with acute leukemia have been documented [1-5] with only 5 pediatric patients. Other authors have reported an increased risk of lymphoma in hemophilia patients, mainly among HIV positive patients [6,7].…”

Non-Hodgkin’s lymphoma in a hemophilic patient with a traumatic hematoma

“…Chemotherapy-induced cytopenia, mucosal toxicity, and hemostatic disturbances may be more problematic than expected. There are only few case reports on the management of leukemia in hemophilic patients [7][8][9]. A study of 2 patients by Zulfikar reported the existence of social and psychological burden associated with the coexistence of 2 difficult diseases, such as acute leukemia and severe hemophilia, among patients and families [7].…”