Letters and Correspondence As soon as the diagnosis was made the patient was started on a sodium bicarbonate infusion. Due to a previous hypersensitivity reaction to allopurinol, this drug was not used. Instead, ibuprofen was employed to reduce uric acid induced renal damage. Prednisone 35 mg twice daily was started the day prior to planned chemotherapy with daunomycin, L-asparaginase, vincristine, and prednisone (LAVP). Twelve hours after prednisone was initiated, the patient became oliguric with evidence of renal failure and serum chemistry as follows: phosphorous 5.92 mmolil, potassium 4.1 mEqil, urea 25.2 mmolil, creatinine 244 pmolil, and uric acid 1,464 p,mol/l. Hemodialysis was initiated the same day and continued for 3 consecutive days after which a rapid recovery of renal function and electrolyte balance occurred. Chemotherapy with the LAVP protocol continued after completion of dialysis along with CNS treatment using cytosine arabinoside and methotrexate via an Ommaya reservoir. A bone marrow aspirate and biopsy, thoracentesis, and CSF analysis done 2 months post-initiation of induction treatment showed no evidence of residual disease. The acute tumor lysis syndrome is a well-recognized complication of cytoreductive therapy for rapidly proliferating neoplasms. This clinical picture is usually seen after chemotherapy is employed in the treatment of non-Hodgkin’s lymphomas and leukemias [ 1 4 1 . Although corticosteroid-induced acute tumor lysis has previously been identified in the non-Hodgkin’s lymphomas, the occurrence of massive tumor lysis in T-cell ALL after corticosteroid administration is not a well-recognized clinical entity. This case, therefore, illustrates the complications that may arise in administering corticosteroids to these patients. The early use of alkaline diuresis and, if necessary, hemodialysis, can however produce excellent clinical results without delaying the initiation of chemotherapy.
67
had been diagnosed. Desferrioxamine chelation treatment had not been suspended during this fever state. At admission the patient appeared well but had a temperature of 39°C. Desferrioxamine treatment was suspended. On the day of admission plasmodium fakiparum-ring forms and gametocytes were identified in thin blood film. Parasitemia was 20,000immc. Chloroquine resistance became manifest but quinine sulphate treatment achieved negative parasitemia 7, 14, and 28 days after beginning the treatment. One of the patient’s blood donors was identified as the infection source. In our case the mild course of the disease for a non-immune subject, despite the delay in diagnosis, was surprising. The mildness of the patient’s clinical picture was not related to her genetic red cell defect because she survived on blood donors’ erythrocytes. The desferrioxamine treatment alone, at 50 mgikglday ( 1 gi24 hr) for 6 daysiweek (not suspended during febrile state until malaria was diagnosed) may have been responsible for an anti-plasmodium falciparum effect in our patient.
SIMONE GANGAROSSA GINOSCHILIRO’ Department of Pediatric Hematology,
ROSARIORusso Department of Infectious Diseases, University of Catania, Catania, Italy
REFERENCES I . Traore 0, Carnevale P, Kaptue-Noche L, M’Bede J , Desfontaine M, Elion J , Labie D, Nagel RL: Preliminary report on the use of desferrioxamine in the
S. RAJAGOPAL J.H. LIPTON 2. H.A. MESSNER Princess Margaret Hospital, Toronto, Ontario
REFERENCES I, Dhingra K , Newcom SR: Acute tumor lysis syndrome in non-Hodgkin lymphoma induccd by dexamethasone. Am J Hematol29: 115-1 16. 1988. 2. Sparano J , Ramircz M , Wiemik PH: Increasing recognition of corticosteroidinduced tumor lysis syndrome in non-Hodgkin’s lymphoma. Cancer 65: 10721073, 1990. 3. Hailer C, Dhadly M: The tumor lysis syndrome. Ann lntem Med I14:808-809, 1991. 4. Loosvcld OJ, Schouten HC, Gaillard CA. Blijham GH: Acute tumor lysis syndrome in a patient with acute lymphoblastic leukemia after a single dose of prednisone. BrJ Haematol 77:122-123, 1991
Desferrioxamine in the Treatment of Plasmodium Falciparum Malaria To the Editor: We read with interest the preliminary report by Traore et al. 111 o n the use of desferrioxamine in the treatment of plasmodium falciparum malaria in humans. Their results ai-e encouraging and agree with results previously obtained in vitro [2,3]. Recently we observed a post-transfusion malaria case in a 7-year-old Italian girl affected by thalassemia major. The patient was admitted to our Division with a 30 day history of fever of unknown origin. She had a slight increase of preexisting hepatosplenomegaly and an increased requirement for blood transfusion. Her general, nutritional, and psychological conditions were satisfactory. Case history was negative for foreign travel. She had been undergoing regular transfusion and iron chelation with desferrioxamine (50 mgikgiday for 6 daysiweek) since thalassemia major
treatment of plasmodium falciparum malaria. Am J Hematol 37:206, 1991. Raventos-Suarez C , Pollack S , Nagel RL: Plasmodium falciparum: Inhibition of in vitro growth by desferrioxamine. Am J Trop Med Hyg 3 I (5):919, 1982. 3. Peto TEA, Hershko C: Iron and infection. In “Iron Chelating Therapy.” Bailliere’s Clinical Haematology, 1989, p 435.
Immune Bernard Soulier-Like Syndrome Associated With Anti-Glycoprotein-lX Antibody To the Editor: We wish to describe a patient who developed a functional platelet defect, mediated by an autoantibody directed specifically to GPIX. Bernard Soulier Syndrome (BSS) is a rare inherited bleeding disorder in which thrombocytopenia with giant platelets and prolonged bleeding time are found [ 11. The platelet defect in BSS has been attributed to the absence of glycoprotein Ib-IX (GP Ib-IX), which serves as a receptor for von Willebrand factor (vWF) [2]. A 75-year-old woman was referred to the hematology clinic for evaluation of thrombocytopenic purpura. Physical examination on admission revealed purpura on both calves and a slightly enlarged liver. Laboratory tests disclosed: Hb I 1 gidl, WBC 3,5OO/kI, a platelet count of 70,OOOikl and a hypercellular bone marrow with numerous (normal and small forms) megakaryocytes. Coagulation tests were normal except for a bleeding time (Dukes) of 8 min (normal range up to 4 min), and a lack of platelet agglutination in response to ristocetin. An increased platelet associated immunoglobulin (PAlg 30 ngi106 platelets, normal range 0-10 ngilO‘ platelets) directed our attention to a possible immune cause for the defect of the patient’s platelets. The combination of pancytopenia and hypercellular bone marrow suggested that this patient had myelodysplastic syndrome (MDS). The relation between the MDS and the immune thrombocytopenic purpura (ITP) remained unsolved. She died 3 years after her first examination due to a sepsis.
67
had been diagnosed. Desferrioxamine chelation treatment had not been suspended during this fever state. At admission the patient appeared well but had a temperature of 39°C. Desferrioxamine treatment was suspended. On the day of admission plasmodium fakiparum-ring forms and gametocytes were identified in thin blood film. Parasitemia was 20,000immc. Chloroquine resistance became manifest but quinine sulphate treatment achieved negative parasitemia 7, 14, and 28 days after beginning the treatment. One of the patient’s blood donors was identified as the infection source. In our case the mild course of the disease for a non-immune subject, despite the delay in diagnosis, was surprising. The mildness of the patient’s clinical picture was not related to her genetic red cell defect because she survived on blood donors’ erythrocytes. The desferrioxamine treatment alone, at 50 mgikglday ( 1 gi24 hr) for 6 daysiweek (not suspended during febrile state until malaria was diagnosed) may have been responsible for an anti-plasmodium falciparum effect in our patient.
SIMONE GANGAROSSA GINOSCHILIRO’ Department of Pediatric Hematology,
ROSARIORusso Department of Infectious Diseases, University of Catania, Catania, Italy
REFERENCES I . Traore 0, Carnevale P, Kaptue-Noche L, M’Bede J , Desfontaine M, Elion J , Labie D, Nagel RL: Preliminary report on the use of desferrioxamine in the
S. RAJAGOPAL J.H. LIPTON 2. H.A. MESSNER Princess Margaret Hospital, Toronto, Ontario
REFERENCES I, Dhingra K , Newcom SR: Acute tumor lysis syndrome in non-Hodgkin lymphoma induccd by dexamethasone. Am J Hematol29: 115-1 16. 1988. 2. Sparano J , Ramircz M , Wiemik PH: Increasing recognition of corticosteroidinduced tumor lysis syndrome in non-Hodgkin’s lymphoma. Cancer 65: 10721073, 1990. 3. Hailer C, Dhadly M: The tumor lysis syndrome. Ann lntem Med I14:808-809, 1991. 4. Loosvcld OJ, Schouten HC, Gaillard CA. Blijham GH: Acute tumor lysis syndrome in a patient with acute lymphoblastic leukemia after a single dose of prednisone. BrJ Haematol 77:122-123, 1991
Desferrioxamine in the Treatment of Plasmodium Falciparum Malaria To the Editor: We read with interest the preliminary report by Traore et al. 111 o n the use of desferrioxamine in the treatment of plasmodium falciparum malaria in humans. Their results ai-e encouraging and agree with results previously obtained in vitro [2,3]. Recently we observed a post-transfusion malaria case in a 7-year-old Italian girl affected by thalassemia major. The patient was admitted to our Division with a 30 day history of fever of unknown origin. She had a slight increase of preexisting hepatosplenomegaly and an increased requirement for blood transfusion. Her general, nutritional, and psychological conditions were satisfactory. Case history was negative for foreign travel. She had been undergoing regular transfusion and iron chelation with desferrioxamine (50 mgikgiday for 6 daysiweek) since thalassemia major
treatment of plasmodium falciparum malaria. Am J Hematol 37:206, 1991. Raventos-Suarez C , Pollack S , Nagel RL: Plasmodium falciparum: Inhibition of in vitro growth by desferrioxamine. Am J Trop Med Hyg 3 I (5):919, 1982. 3. Peto TEA, Hershko C: Iron and infection. In “Iron Chelating Therapy.” Bailliere’s Clinical Haematology, 1989, p 435.
Immune Bernard Soulier-Like Syndrome Associated With Anti-Glycoprotein-lX Antibody To the Editor: We wish to describe a patient who developed a functional platelet defect, mediated by an autoantibody directed specifically to GPIX. Bernard Soulier Syndrome (BSS) is a rare inherited bleeding disorder in which thrombocytopenia with giant platelets and prolonged bleeding time are found [ 11. The platelet defect in BSS has been attributed to the absence of glycoprotein Ib-IX (GP Ib-IX), which serves as a receptor for von Willebrand factor (vWF) [2]. A 75-year-old woman was referred to the hematology clinic for evaluation of thrombocytopenic purpura. Physical examination on admission revealed purpura on both calves and a slightly enlarged liver. Laboratory tests disclosed: Hb I 1 gidl, WBC 3,5OO/kI, a platelet count of 70,OOOikl and a hypercellular bone marrow with numerous (normal and small forms) megakaryocytes. Coagulation tests were normal except for a bleeding time (Dukes) of 8 min (normal range up to 4 min), and a lack of platelet agglutination in response to ristocetin. An increased platelet associated immunoglobulin (PAlg 30 ngi106 platelets, normal range 0-10 ngilO‘ platelets) directed our attention to a possible immune cause for the defect of the patient’s platelets. The combination of pancytopenia and hypercellular bone marrow suggested that this patient had myelodysplastic syndrome (MDS). The relation between the MDS and the immune thrombocytopenic purpura (ITP) remained unsolved. She died 3 years after her first examination due to a sepsis.
