Paroxysmal Nocturnal Hemoglobinuria Associated With the Acquired Immunodeficiency Syndrome

Baumann, Michael A.; Pacheco, J.; Paul, Cassandra C.; Sorg, Timothy B.; Hillman, N

doi:10.1001/archinte.1988.00380010214023

Cited by 6 publications

(3 citation statements)

References 7 publications

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“…Interestingly, reticulocytopenia appeared to be commonly found among HIV patients with autoimmune hemolytic anemia [90]. Rarely, cases of Marchiafava's anemia [7] and hemolytic uremic syndromes [31] have been reported, but there is no evidence that HIV patients are overall at a higher risk for these types of anemia. However, overt hemolysis is extremely rare in this population [26].…”

Section: Hemolysismentioning

confidence: 99%

Pathogenesis and pathophysiology of anemia in HIV infection

Kreuzer

Rockstroh

1997

Annals of Hematology

109

100

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Anemia occurs frequently among patients seropositive for human immunodeficiency virus (HIV), but its multifactorial origin complicates its differential diagnosis and adequate treatment. In addition, the etiology of anemia in HIV infection often remains unclear. In recent years several attempts have been undertaken to elucidate the mechanisms leading to HIV-associated anemia. Direct infection of erythroid progenitors has been discussed, but could not be proven. Furthermore, soluble factors like HIV proteins and cytokines have been suggested to inhibit growth of hematopietic cells in the bone marrow of HIV-infected patients. However, so far no statements can be made whether these factors are directly involved in myelosuppression or mediate their effect by inhibiting growth-factor synthesis. Opportunistic complications represent the underlying cause for anemia in a large number of HIV-infected patients. Next to this rather obvious reason for anemia, iatrogenic anemia induced by myelosuppressive drugs is also very common. It is of note, however, that modern dosages of < 600 mg zidovudine (ZDV) daily rarely cause anemia. Instead, other drugs that can induce anemia itself or by enhancing ZDV plasma concentrations must be considered important contributing factors. Deficiency of vitamin B12, folate and iron are frequently reported in HIV patients. However, specific investigations revealed appropriate storage amounts of these micronutrients. Supplementation may be beneficial in some patients, but often fails to reverse anemia in this population. In anemic HIV patients reticulocytopenia is a consistent finding. Additionally, inadequately low endogenous erythropoietin concentrations have been repeatedly reported. Thus, it is speculated that a blunted erythropoietin feedback mechanism contributes substantially to the pathogenesis of anemia in HIV patients.

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Section: Hemolysismentioning

confidence: 99%

Pathogenesis and pathophysiology of anemia in HIV infection

Kreuzer

Rockstroh

1997

Annals of Hematology

109

100

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“…Eight of nine patients transplanted have become longterm survivors with follow-up ranging from more than 2.2 years to greater than 19.1 years. Karnofsky performance scores are 100 (patients I , 3,4,7,9), 90 (patient S), and 80 (patients 2, 6). Except for patient 7 noted above, the hemograms of these patients are normal and none required blood product support.…”

Section: Characteristics Of Patients Transplanted For Pnhmentioning

confidence: 99%

“…PNH, in rare patients, may also occur concomitantly with myelodysplastic syndrome (MDS) or acute nonlymphocytic leukemia [8]. A PNH-like syndrome was recently reported in a patient with human immunodeficiency virus (HIV) infection [9].…”

Section: Introductionmentioning

confidence: 99%

Marrow transplantation for paroxysmal nocturnal hemoglobinuria

1992

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Between 1971 and 1990, nine patients ranging in age from 14-38 years received marrow transplants for paroxysmal nocturnal hemoglobinuria (PNH). Six were transplanted for aplastic complications of PNH. Four of these were from HLA-identical siblings, and the patients were conditioned with cyclophosphamide. One graft was form a syngeneic twin without conditioning, and one from a two HLA-antigen nonidentical father after conditioning with cyclophosphamide and total body irradiation. Three of the four recipients of allogeneic marrow developed acute and two chronic graft-versus-host disease (GVHD). Five of six transplanted for severe aplastic anemia are long-term survivors with follow-up ranging from more than 6.2 to more than 19.1 years. The HLA nonidentical transplant recipient experienced graft rejection and died of a pulmonary hemorrhage. Three patients were transplanted for nonaplastic complications of PNH consisting of life threatening recurrent thromboses or refractory hemolysis. Two of these patients received marrow grafts from HLA-identical siblings after conditioning with busulfan and cyclophosphamide. They are surviving with normal hemograms greater than 2.2 and greater than 2.5 years and had mild chronic GVHD which resolved, although one has biochemical evidence of PNH in 15% of the red cells. One received a syngeneic marrow graft without conditioning but reverted to PNH. He is alive greater than 8.6 years after transplantation. Marrow transplantation for aplastic complications of PNH is successful, well tolerated, and compatible with long-term survival when an HLA-identical sibling or a syngeneic donor is available. For patients without aplasia, one must weigh the complications of transplantation with the life threatening nature of thrombotic episodes and hemolysis.

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P

Lachman

2007

Taybi and Lachman's Radiology of Syndromes, Metabolic Disorders and Skeletal Dysplasias

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Paroxysmal Nocturnal Hemoglobinuria Associated With the Acquired Immunodeficiency Syndrome

Cited by 6 publications

References 7 publications

Pathogenesis and pathophysiology of anemia in HIV infection

Pathogenesis and pathophysiology of anemia in HIV infection

Marrow transplantation for paroxysmal nocturnal hemoglobinuria

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