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PubMed2006

Catheter-directed thrombolysis and thrombectomy for the Budd-Chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.

Adolescent↗

PubMed2006

[Acute renal failure secondary to paroxysmal cold hemoglobinuria].

Paroxysmal cold hemoglobinuria is a type of hemolytic anemia that mainly affects children. Tubular renal injury induced by the heme pigment in intravascular hemolysis is a rare cause of renal failure. We describe the case of a 5-year-old boy who presented with dehydration and dark urine a few hours after exposure to cold. The child had had an upper respiratory infection the previous week. He developed anemia and acute renal failure. The direct antiglobulin test was positive with anti-C3c and C3d. The diagnosis of paroxysmal cold hemoglobinuria was confirmed by the presence of biphasic antibody in the Donath-Landsteiner test.

Acute Kidney Injury↗

PubMed1982

Circulating immune complexes in paroxysmal nocturnal hemoglobinuria.

Circulating immune complexes were investigated in sera of 13 patients with paroxysmal nocturnal hemoglobinuria. A significant inhibition was observed in the complement-dependent rosette formation (EAC) in 6 cases; 2 patients presented an increased anticomplementary activity; antilymphocyte antibodies were demonstrated in 2 patients. The levels of C3d and immunoconglutinins were also increased; there were no differences in the total hemolytic complement between patients and normal controls. There was a significant correlation between C3d and immunoconglutinins with the inhibition of the EAC rosette formation test. These results provide evidence for the presence of circulating immune complexes in paroxysmal nocturnal hemoglobinuria.

Adult↗

PubMed1990

Neonatal ABO incompatibility. Complicated by hemoglobinuria and acute renal failure.

The authors present two infants with isoimmune hemolytic disease due to ABO incompatibility complicated by massive hemoglobinuria and secondary acute renal failure. This represents an incidence of 0.36% of all neonates with ABO hemolytic disease in the author's newborn population. Only two patients have been reported previously to have similar complications. Analysis of data of these four infants revealed the clinical characteristics of this complication of ABO incompatibility: 1) very low frequency; 2) early onset of hemoglobinuria (first voided urine) and of acute renal failure (first 2 days of life); 3) lack of correlation between the clinical presentation of hemolytic disease and appearance and severity of renal failure; 4) complete recovery of renal functions following intravenous fluid administration; and 5) normal renal radiologic investigations.

ABO Blood-Group System↗

PubMed2005

Disseminated intravascular coagulation associated with acute hemoglobinemia or hemoglobinuria following Rh(0)(D) immune globulin intravenous administration for immune thrombocytopenic purpura.

The Food and Drug Administration (FDA) licensed Rh(o)(D) immune globulin intravenous (anti-D IGIV) on March 24, 1995, for treatment of immune thrombocytopenic purpura (ITP). A previous review described data on 15 patients who experienced acute hemoglobinemia or hemoglobinuria following anti-D IGIV administration for ITP or secondary thrombocytopenia. Eleven of those patients also experienced clinically compromising anemia, transfusion with packed red blood cells, renal insufficiency, dialysis, or death. That review suggested that patients receiving anti-D IGIV be monitored for those and other potential complications of hemoglobinemia, particularly disseminated intravascular coagulation (DIC). Through November 30, 2004, the FDA received 6 reports of DIC associated with "acute hemolysis" (or similar terms), 5 of which involved fatalities. The attending or consulting physicians assessed that acute hemolysis or DIC caused or contributed to each death. This review presents the first case series of DIC associated with acute hemoglobinemia or hemoglobinuria following anti-D IGIV administration for ITP. The purpose of this review is to increase awareness among physicians and other health care professionals that DIC may be a rare but potentially severe complication of anti-D IGIV treatment. Increased awareness of DIC as a diagnostic possibility may enable prompt recognition and medical intervention in affected patients.

Acute Disease↗

PubMed2005

Sustained response and long-term safety of eculizumab in paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by clonal expansion of red blood cells (RBCs) lacking the ability to inhibit complement-mediated hemolysis. Eculizumab, a humanized monoclonal antibody that binds the C5 complement protein, blocks serum hemolytic activity. This study evaluated the long-term safety and efficacy of eculizumab in 11 patients with PNH during an open-label extension trial. After completion of an initial 12-week study, all patients chose to participate in the 52-week extension study. Eculizumab, administered at 900 mg every 12 to 14 days, was sufficient to completely and consistently block complement activity in all patients. A dramatic reduction in hemolysis was maintained throughout the study, with a decrease in lactate dehydrogenase (LDH) levels from 3110.7 IU/L before treatment to 622.4 IU/L (P = .002). The proportion of PNH type III RBCs increased from 36.7% at baseline to 58.4% (P = .005). The paroxysm rate of days with gross evidence of hemoglobinuria per patient each month decreased from 3.0 during screening to 0.2 (P < .001) during treatment. The median transfusion rate decreased from 1.8 U per patient each month before eculizumab treatment to 0.3 U per patient each month (P = .001) during treatment. Statistically significant improvements in quality-of-life measures were also maintained during the extension study. Eculizumab continued to be safe and well tolerated, and all patients completed the study. The close relationship between sustained terminal complement inhibition, hemolysis, and symptoms was demonstrated.

Antibodies, Monoclonal↗

PubMed1993

Cerebral venous thrombosis in paroxysmal nocturnal hemoglobinuria: report of two cases.

We report the clinical, radiologic, and postmortem findings in two patients with paroxysmal nocturnal hemoglobinuria (PNH) who developed cerebral venous thromboses (CVTs). In contrast with those in most published cases, our patients did not have focal neurologic signs. Antemortem diagnosis of CVT had been made by MR cerebral venograms. We conclude that (1) PNH should be considered in any patient with stroke associated with iron deficiency anemia, hemolysis, hemoglobinuria, or hemosiderinuria; (2) PNH should be in the differential diagnosis of CVT; (3) the latter could present without focal neurologic signs; and (4) MR cerebral venography may be a reliable diagnostic alternative to cerebral angiography when CVT is suspected.

Cerebral Angiography↗

PubMed1975

Renal impairment in patients with paroxysmal nocturnal hemoglobinuria.

Three patients with paroxysmal nocturnal hemoglobinuria accompanied by chronic renal lesions were studied. All the cases had histories of severe hemolytic anemia and repeated hemoglobinuria. The biopsy specimen of the kidney of two patients (Case 1 and Case 2) showed interstitial nephritis. Renal glucosuria, tubular proteinuria, increased urate clearance (Case 2) and reduced tubular reabsorption of phosphate (Case 3) were revealed in Case 2 and Case 3, suggesting renal tubular impairment. From the nephrological point of view, hemodynamic alteration resulting from intravascular hemolysis and severe persistent chronic anemia may primarily be responsible for the renal impairment.

Adult↗

PubMed1988

Periodontal surgery for a patient with paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon blood stem cell disorder. The acquired defect is in the cell membrane of the precursor of the affected clone, which renders erythrocytes, platelets and granulocytes unusually sensitive to the hemolytic effects of complement. It is characterized by chronic hemolysis, intermittent hemoglobinuria, thrombotic events and bone marrow hypoplasia. Hemolysis is precipitated by infection, strenuous exercise, surgery or menstruation. The dilemma of managing a PNH patient is that both the periodontal infection and the surgical treatment are precipitating factors. Management is further complicated by the fact that the patient is receiving corticosteroids. The treatment of the PNH patient is complex and potentially hazardous. It requires close cooperation between the periodontist and the hematologist. This paper describes the management of one such case.

Adult↗

PubMed1984

Papilledema in paroxysmal nocturnal hemoglobinuria.

A 26-year-old man with the diagnosis of paroxysmal nocturnal hemoglobinuria presented with papilledema due to increased intracranial pressure without localizing neurological signs. It is concluded that papilledema and benign intracranial hypertension could result from a cerebral vein thrombosis which can complicate the course of paroxysmal nocturnal hemoglobinuria. This has not been a well-recognized feature of the disease.

Adult↗

PubMed1985

Thrombolytic therapy for inferior vena cava thrombosis in paroxysmal nocturnal hemoglobinuria.

Two patients with paroxysmal nocturnal hemoglobinuria had increasing abdominal girth and ascites. The Budd-Chiari syndrome or inferior vena cava thrombosis was shown by angiography. After thrombolytic therapy, both patients improved, and thrombolysis was shown by radiography. Neither patient had induction of hemolysis secondary to these agents. These studies suggest that both streptokinase and urokinase are safe and effective in the treatment of intra-abdominal venous thromboses associated with paroxysmal nocturnal hemoglobinuria.

Abdomen↗

PubMed1999

Paroxysmal nocturnal hemoglobinuria cells in patients with bone marrow failure syndromes.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem-cell disorder in which the affected cells are deficient in glycosylphosphatidylinositol (GPI)-anchored proteins. Paroxysmal nocturnal hemoglobinuria is frequently associated with aplastic anemia, although the basis of this relation is unknown. OBJECTIVE: To assess the PNH status of patients with diverse marrow failure syndromes. DESIGN: Correlation of cytofluorometric data with clinical features. SETTING: Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland. PATIENTS: 115 patients with aplastic anemia, 39 patients with myelodysplasia, 28 patients who had recently undergone bone marrow transplantation, 18 patients with cancer that was treated with chemotherapy, 13 patients with large granular lymphocytosis, 20 controls who had received renal allografts, and 21 healthy participants. INTERVENTION: Patients with aplastic anemia, myelodysplasia, or renal allografts received antithymocyte globulin. MEASUREMENTS: Flow cytometry was used to assess expression of GPI-anchored proteins on granulocytes. RESULTS: Evidence of PNH was found in 25 of 115 (22%) patients with aplastic anemia. No patient with normal GPI-anchored protein expression at presentation developed PNH after therapy (n = 16). Nine of 39 (23%) patients with myelodysplasia had GPI-anchored protein-deficient cells. Abnormal cells were not detected in patients with constitutional or other forms of bone marrow failure or in renal allograft recipients who had received antithymocyte globulin. Aplastic anemia is known to respond to immunosuppressive therapy; in myelodysplasia, the presence of a PNH population was strongly correlated with hematologic improvement after administration of antithymocyte globulin (P = 0.0015). CONCLUSIONS: Flow cytometric analysis is superior to the Ham test and permits concomitant diagnosis of PNH in about 20% of patients with myelodysplasia (a rate similar to that seen in patients with aplastic anemia). The presence of GPI-anchored protein-deficient cells in myelodysplasia predicts responsiveness to immunosuppressive therapy. Early emergence of GPI-anchored protein-deficient hematopoiesis in a patient with marrow failure may point to an underlying immune pathogenesis.

Antilymphocyte Serum↗

PubMed1976

Paroxysmal nocturanal hemoglobinuria and marrow failure treated by infusion of marrow from an identical twin.

An 18-year-old white Canadian male patient with paroxysmal nocturnal hemoglobinuria and refractory marrow failure received a marrow infusion from his normal identical twin brother without prior ablation of the patient's marrow by drugs or irradiation. After 2 years of follow-up, the patient is well with no evidence of clinical disease or significant hematological abnormality. The results suggest that normal marrow stem cells can have a selective advantage over the abnormal paroxysmall nocturnal hemoglobinuria clone.

Adolescent↗

PubMed2001

[Recurrent acute myocardial infarction in a patient with nocturnal paroxysmal hemoglobinuria].

Paroxysmal nocturnal hemoglobinuria is a form of acquired hemolytic anemia with a high incidence of thrombotic complications, generally in the venous district; arterial thrombosis is rare, and exceptional in the coronary tree. We describe the case of a man who had two episodes of myocardial infarction, both during a hemoglobinuric crisis; this patient was free from cardiovascular risk factors and angiography revealed that there was no coronary stenosis. The clinical course was favorable and the patient's response to thrombolytic and dicumarol therapy was satisfactory. To our knowledge, this is the second case of coronary involvement in paroxysmal nocturnal hemoglobinuria described in the literature.

Hemoglobinuria, Paroxysmal↗

PubMed1992

The receptor for urokinase-type plasminogen activator is deficient on peripheral blood leukocytes in patients with paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal defect in bone marrow-derived cells and is clinically associated with intravascular hemolysis, hemoglobinuria, and an increased frequency of venous thrombosis. The common denominator of PNH-affected blood cells appears to be a defect in the membrane attachment of proteins normally anchored by glycosyl-phosphatidylinositol (GPI). We report here that the cellular receptor for urokinase-type plasminogen activator (u-PAR) is deficient on affected peripheral blood monocytes and granulocytes from four individuals with PNH as evidenced by chemical cross-linking analysis as well as by immunofluorescence flow cytometry using a monoclonal anti-u-PAR antibody. In contrast, on normal blood monocytes and granulocytes we find significant amounts of u-PAR, which is attached to the plasma membrane by a GPI-anchor as defined by its sensitivity towards a specific phospholipase treatment. By two-color flow cytometry it was shown that deficiency of u-PAR expression paralleled that of another GPI-anchored protein. As u-PAR is involved in the initiation of pericellular proteolysis, the reduced expression of u-PAR on PNH-affected leukocytes led to an overall reduction in the capacity for plasminogen activation by cell-surface-bound urokinase. Whereas the abnormal susceptibility of PNH-affected erythrocytes to lysis by autologous complement has been related to the low expression of three GPI-anchored complement regulatory proteins on the cell surface, we now propose that lack of u-PAR expression on the surface of peripheral blood leukocytes may be causally related to the high incidence of venous thrombosis observed in PNH patients.

Animals↗

PubMed2003

[Paroxysmal nocturnal hemoglobinuria associated with intestinal ischemia leading to small bowel perforation].

Paroxysmal nocturnal hemoglobinuria is rarely associated with intestinal complications. We report a case of paroxysmal nocturnal hemoglobinuria with small bowel ischemia leading to ileal perforation. In the literature, an ulcerative jejuno-ileitis has been reported in 6 cases of intestinal ischemia, due to thrombosis of mucosal small vessels. This disease is usually revealed by abdominal pain. Based on published cases and our observation of intestinal ischemia leading to small bowel perforation, surgery should be considered as the first-line treatment, especially when small bowel lesions are limited.

Adult↗

PubMed2004

Paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder resulting from a somatic mutation in the hematopoietic stem cell. It is characterized by intravascular hemolysis, cytopenias, frequent infections, bone marrow hypoplasia, and a high incidence of life-threatening venous thrombosis. An absent glycosylphosphatidylinositol (GPI)-anchored receptor prevents several proteins from binding to the erythrocyte membrane. These include the complement-regulatory proteins, CD55 and CD59, whose absence results in enhanced complement-mediated lysis. Patients present with anemia and hemoglobinuria. Laboratory diagnosis includes the sucrose hemolysis test, Ham acid hemolysis test, and fluorescent-activated cell analysis. There is considerable overlap between PNH, aplastic anemia, and myelodysplastic syndrome and some cases evolve into acute leukemia. Treatment is mainly supportive consisting of transfusion therapy, anticoagulation, and antibiotic therapy. Hematopoietic stem cell transplantation may be curative.

Anemia↗

PubMed1997

[Study on prethrombotic state in patients with paroxysmal nocturnal hemoglobinuria].

OBJECTIVE: To explore the mechanism of thrombogenesis in patients with paroxysmal nocturnal hemoglobinuria (PNH). METHODS: Thirteen parameters concerning endotheliocytes, platelets, blood coagulation, and fibrinolysis were measured in 21 PNH patients. RESULTS: As compared with normal controls, plasma fibrin peptide A (FPA) levels were significantly higher, but antithrombin-III (AT-III) and protein C (PC) antigen levels lower in the PNH patients. Plasma tissue plasminogen activator (t-PA) activities were significantly lower while plasminogen activator inhibitor (PAI) activities markedly higher in the patients than those in the controls. The levels of the blood coagulation and fibrinolysis parameters changed more notably during the occurrence of thrombosis and hemoglobinuria episodes in the PNH patients. Platelet adhesion rates, platelet aggregation rates, beta-thromboglobulin (beta-TG), and granule membrane protein-140 (GMP-140) were within the normal ranges. Plasma vWF:Ag and endothelin-1 (ET-1) concentrations were normal in the patients as well. CONCLUSION: Prethrombotic state is present in PNH patients, which may be caused by hypercoagulability and low fibrinolytic activity.

Adult↗