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PubMed1983

Increased sensitivity to complement of erythroid and myeloid progenitors in paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria is an acquired hemolytic anemia characterized by a membrane defect leading to increased sensitivity of erythrocytes, granulocytes, platelets, and bone-marrow erythroid and myeloid cells to complement-mediated lysis. To determine whether the phenotype of paroxysmal nocturnal hemoglobinuria is also expressed on erythroid and myeloid progenitors, marrow cells from five patients with the disease were exposed to a sucrose hemolytic system and then assayed for colony-forming units-erythroid (CFU-E), burst-forming units-erythroid (BFU-E), and colony-forming units-granulocyte/macrophage (CFU-GM). A 50 percent or greater decrease in the numbers of erythroid and myeloid colonies was noted when marrow cells from the patients with paroxysmal nocturnal hemoglobinuria were exposed to a sucrose solution of low ionic strength in the presence of complement but not in its absence. Such a decrease was not noted in similarly treated normal marrow cells or in marrow cells from a patient with the disease in remission. These results suggest that in paroxysmal nocturnal hemoglobinuria, CFU-E, BFU-E, and CFU-GM express a membrane abnormality similar to that on erythrocytes, and that the disease is the result of a change occurring at the level of the pluripotent hematopoietic stem cell.

Adult↗
PubMed1985

Deficiency of the complement regulatory protein, "decay-accelerating factor," on membranes of granulocytes, monocytes, and platelets in paroxysmal nocturnal hemoglobinuria.

Erythrocytes from patients with paroxysmal nocturnal hemoglobinuria are deficient in decay-accelerating factor, a membrane protein that inhibits the complement C3 convertases. We studied the expression of this protein on leukocytes and platelets from four patients with paroxysmal nocturnal hemoglobinuria, using cytofluorographic analysis and antibody to decay-accelerating factor. The granulocytes and monocytes had a bimodal distribution of fluorescence, indicating antigen-deficient and antigen-positive subpopulations of cells. In contrast, granulocytes and monocytes from normal donors and patients with other diseases had no antigen-deficient cells. Platelets from the four patients with paroxysmal nocturnal hemoglobinuria had less fluorescence than normal platelets. Furthermore, surface-radiolabeled granulocytes and platelets from one of the four patients, which were maximally deficient in decay-accelerating factor, also lacked antigen that was immunoprecipitable by specific antibody to this protein. Thus, paroxysmal nocturnal hemoglobinuria is a clonal disorder characterized by deficient membrane expression of decay-accelerating factor on granulocytes, monocytes, and platelets, as well as on erythrocytes.

Adult↗
PubMed1975

Paroxysmal cold hemoglobinuria. Transmission and scanning electron microscopy features of erythrocytes.

Djaldetti, Meir, Elion, Dorith, Bessler, Hana, and Fishman, Pnina: Paroxysmal cold hemoglobinuria. Tranmission and scanning electron microscopy features of erythrocytes. Am J Clin Pathol 63. 804-810, 1975. The case history of a patint with paroxysmal cold hemoglobinuria of the chronic iduipathic type is reported. The patient showed a marked dependency on the external temperature, with severe hemolytic anemia and hemoglobinuria during the winter. Examination of the erythrocytes with the transmission electron microscope showed abnormal forms identified as ropalocytes, whereas examination with the scanning electron microscope revealed "holes" in the erythrocytes. (Key words: Paroxysmal cold hemoglobinuria' Ultrastructure of erythrocytes.

Adult↗
PubMed1981

Paroxysmal nocturnal hemoglobinuria: small bowel findings.

Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic hemolytic anemia of unknown etiology associated with morning hemoglobinuria, variable acholuric jaundice, and striking hemosiderinuria, all signs of hemolysis. Paroxysmal nocturnal hemoglobinuria with infarction and hemorrhage of the gut leading to emergency surgery has been reported [1]; a history of thrombosis may be found before PNH is diagnosed [2]. We describe the radiographic findings in the small bowel of a patient with paroxysmal nocturnal hemoglobinuria during an episode of moderately severe abdominal symptoms. Ours is the second reported case using barium study.

Adult↗
PubMed2000

Acute onset hemoglobinemia and/or hemoglobinuria and sequelae following Rh(o)(D) immune globulin intravenous administration in immune thrombocytopenic purpura patients.

Rh(o)(D) immune globulin intravenous (anti-D IGIV) was licensed by the United States Food and Drug Administration (FDA) in March 1995 to treat patients with immune thrombocytopenic purpura (ITP). Anti-D IGIV induces extravascular hemolysis, an expected adverse reaction that is consistent with the presumed mechanism of action. Between licensure and April 1999, the FDA received 15 reports of hemoglobinemia and/or hemoglobinuria following anti-D IGIV administration that met the case definition for this review. The mechanism responsible for hemoglobinemia and/or hemoglobinuria is unexplained. Review of these reports was prompted by the seriousness and the unexpectedness of treatment-associated sequelae experienced by 11 patients. Of these patients, 7 developed sufficient onset or exacerbation of anemia that orders were written for packed red blood cell transfusions, although only 6 patients were transfused. Eight patients experienced the onset or exacerbation of renal insufficiency, and 2 patients underwent dialysis. One patient died due to complications of exacerbated anemia. Six patients experienced 2 to 3 sequelae. Absent validated incidence data, a 1.5% estimated incidence rate from published clinical trial data and a 0.1% estimated reporting rate from FDA and drug utilization data were calculated for reported cases of hemoglobinemia and/or hemoglobinuria. This review presents the first case series of anti-D-IGIV-associated hemoglobinemia and/or hemoglobinuria and provides pretreatment and posttreatment clinical and laboratory findings of the case series patients. The primary purpose of this review is to increase awareness of this potentially serious occurrence among physicians and health care professionals who manage ITP patients treated with anti-D IGIV, thereby enabling prompt recognition and treatment of sequelae. (Blood. 2000;95:2523-2529)

Adolescent↗
PubMed2004

Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) arises from a somatic mutation of the PIG-A gene in a hematopoietic stem cell and the subsequent production of blood cells with a deficiency of surface proteins that protect the cells against attack by the complement system. We tested the clinical efficacy of eculizumab, a humanized antibody that inhibits the activation of terminal complement components, in patients with PNH. METHODS: Eleven transfusion-dependent patients with PNH received infusions of eculizumab (600 mg) every week for four weeks, followed one week later by a 900-mg dose and then by 900 mg every other week through week 12. Clinical and biochemical indicators of hemolysis were measured throughout the trial. RESULTS: Mean lactate dehydrogenase levels decreased from 3111 IU per liter before treatment to 594 IU per liter during treatment (P=0.002). The mean percentage of PNH type III erythrocytes increased from 36.7 percent of the total erythrocyte population to 59.2 percent (P=0.005). The mean and median transfusion rates decreased from 2.1 and 1.8 units per patient per month to 0.6 and 0.0 units per patient per month, respectively (P=0.003 for the comparison of the median rates). Episodes of hemoglobinuria were reduced by 96 percent (P<0.001), and measurements of the quality of life improved significantly. CONCLUSIONS: Eculizumab is safe and well tolerated in patients with PNH. This antibody against terminal complement protein C5 reduces intravascular hemolysis, hemoglobinuria, and the need for transfusion, with an associated improvement in the quality of life in patients with PNH.

Adult↗
PubMed1984

Intestinal infarction caused by paroxysmal nocturnal hemoglobinuria.

A patient with paroxysmal nocturnal hemoglobinuria of 14 years duration presented with severe abdominal pain and fever. On admission, his hematocrit had fallen to 19% from his usual level of approximately 30%, and stools were positive for occult blood. Dilated loops of small bowel with air fluid levels were noted on radiographs, and sonography revealed free intraperitoneal fluid. Several sections of gangrenous jejunum and ileum were resected at exploratory laparotomy, and mesenteric venous thromboses were present. This is the second report of pathologically proven intestinal infarction in a patient with paroxysmal nocturnal hemoglobinuria who has survived this abdominal emergency with surgical intervention. Although abdominal pain in patients with paroxysmal nocturnal hemoglobinuria has frequently been attributed to mesenteric venous thromboses, this has rarely been documented either during life or at autopsy.

Hemoglobinuria, Paroxysmal↗
PubMed1999

Purification, properties, and amino acid sequence of a hemoglobinuria-inducing phospholipase A(2), MiPLA-1, from Micropechis ikaheka venom.

Dark-colored urine is one of the clinical symptoms of envenomation by Micropechis ikaheka (New Guinea small-eyed snake). We have purified a phospholipase A(2), MiPLA-1, which induces dark-colored urine in experimental mice, to homogeneity. The analysis of the dark-colored urine by electrophoresis and N-terminal sequence determination indicated that the color of mouse urine is due to hemoglobin in the urine but not myoglobin. MiPLA-1 is the first hemoglobinuria-inducing toxin. Insignificant hemolytic activity of MiPLA-1 indicates that hemoglobinuria is not due to lysis of erythrocytes by MiPLA-1. This suggests that hemoglobinuria induced by MiPLA-1 may be due to kidney leakage caused by unknown mechanisms. MiPLA-1 also showed other biological effects, including myotoxicity as well as anticoagulant and antiplatelet effects. Structural studies show that MiPLA-1 is a basic protein with a molecular mass of 14041.60 +/- 1.78 as determined by electrospray mass spectrometry. We have determined the complete amino acid sequence of MiPLA-1. It is a 124-amino-acid protein with a "pancreatic loop" and belongs to group IB phospholipase A(2) enzymes. Two short segments flanked by proline brackets are found in the sequence of MiPLA-1. These segments are on the surface of the molecule and hence may be involved in protein-protein recognition.

Amino Acid Sequence↗
PubMed1980

Prevention of hemoglobinuria by administration of haptoglobin.

This report describes the role of haptoglobin in preventing hemoglobinuria. In rabbits, human haptoglobin administered i.v. worked preventively against the development of hemoglobinuria in spite of the presence of a large amount of hemolysate containing free hemoglobin. More specifically: (1) the kidney did not present a dark brown color, (2) the deposition of iron in the kidney was significantly less than that in the same organ of rabbits given hemolysate only, and (3) the microangiographic and histological findings of the kidney were close to normal patterns. From the above it may be concluded that the administration of haptoglobin is effective for the prevention of hemoglobinuria and subsequent renal damage.

Angiography↗
PubMedDate not supplied

Widespread abdominal venous thrombosis in paroxysmal nocturnal hemoglobinuria diagnosed on CT.

Paroxysmal nocturnal hemoglobinuria is a rare disorder characterized by pancytopenia. One of the main manifestations of this disease is development of widespread life-threatening venous thrombosis, which may involve multiple abdominal veins. We describe two patients with paroxysmal nocturnal hemoglobinuria with clinically unsuspected portal, splenic, and mesenteric venous thromboses that were diagnosed on computed tomography. This complication should be clinically suspected in patients suffering from paroxysmal nocturnal hemoglobinuria who present with abdominal pain, and it should be sought by the radiologist on pre- and postcontrast computed tomography.

Abdomen↗
PubMed2004

Paroxysmal nocturnal hemoglobinuria in a girl with hemolysis and "hematuria".

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder of unknown frequency. In its classic form, PNH is characterized by hemolysis accompanied by nocturnal hemoglobinuria. The clinical course is unpredictable and may vary from severe hemolysis and recurrent venous thrombosis to latent periods with milder symptoms. We report a 15-year-old girl with hemolytic episodes, abdominal pain, and passage of dark urine. Hemoglobinuria was demonstrated by a "blood"-positive dipstick test in the absence of red blood cells in the urinary sediment. The diagnosis of PNH was confirmed by flow cytometry.

Adolescent↗
PubMed1995

Cholangitis associated with paroxysmal nocturnal hemoglobinuria: another instance of ischemic cholangiopathy?

Ischemia is increasingly recognized as a cause of cholangiopathy. The aim of this study was to report a case of association of paroxysmal nocturnal hemoglobinuria with abrupt-onset cholangiopathy. Anomalies resembling sclerosing cholangitis were documented in a patient suffering from recurrent biliary pain. None of the conditions that have been associated with primary sclerosing cholangitis or other forms of cholangiopathy was present, but shortly thereafter, paroxysmal nocturnal hemoglobinuria occurred. Hepatic vein thrombosis later complicated the course of the disease. Because the fortuitous coincidence of these uncommon conditions is unlikely, this case indicates that paroxysmal nocturnal hemoglobinuria is a cause of ischemic cholangiopathy. Other thrombogenic conditions may also be implicated in some instances of apparently idiopathic cholangiopathy.

Adult↗
PubMed1996

In vivo effects of various therapies on complement-sensitive erythrocytes in paroxysmal nocturnal hemoglobinuria.

The percentage of complement-sensitive erythrocytes varies among patients with paroxysmal nocturnal hemoglobinuria (PNH) and is related to disease severity. We examined the changes of complement-sensitive erythrocytes following administration of androgens, prednisolone, dextran, and iron to 12 PNH patients using the complement lysis sensitivity test or flow cytometric analysis of decay accelerating factor and CD59/membrane attack complex-inhibitory factor expression for 11 years. Five untreated PNH patients were also studied as a control group. The complement-sensitive erythrocyte count remained almost constant in the control group, while it increased in four out of five patients receiving androgens. In addition, it decreased in two out of three patients receiving prednisolone, increased in both patients treated with dextran, and increased slightly in two of the three patients receiving iron therapy. Episodes of hemoglobinuria increased in three of the nine patients showing an increase of complement-sensitive erythrocytes, and decreased in four patients receiving prednisolone or dextran. A good response to treatment was clinically observed in four patients receiving androgens, in one patient treated with prednisolone, and in one patient receiving dextran according to the scoring system. These findings suggest that PNH remains stable when the number of complement-sensitive erythrocytes remains fairly constant, and that the PNH III erythrocyte count is especially related to the frequency of hemoglobinuria. Thus, it seems to be important to determine the long-term effect of drug therapy on complement-sensitive erythrocytes to select the most appropriate treatment.

Adolescent↗
PubMed2003

Paroxysmal cold hemoglobinuria and cardiopulmonary bypass.

Paroxysmal cold hemoglobinuria, a cold-reactive autoimmune disease associated with the Donath-Landsteiner antibody, has not been described in patients undergoing cardiac surgery. We report a case of mitral valve replacement in a woman with a positive Donath-Landsteiner antibody and a history of recurrent hemolysis and hemoglobinuria secondary to cold exposure. Successful perioperative management is described, as is a discussion of paroxysmal cold hemoglobinuria.

Cardiopulmonary Bypass↗
PubMed1975

Urologic manifestations of paroxysmal nocturnal hemoglobinuria.

A long-term study of 17 patients with paroxysmal nocturnal hemoglobinuria revealed an unexpectedly high incidence of functional and anatomic renal abormalities. All patients demonstrated varying degrees of hematuria and proteinuria distinct from hemoglobinuria. All patients also had granular casts in multiple urinalyses. Evaluation of renal function revealed hyposthenuria, abnormal tubular function and declining creatinine clearance. Radiologically, one or more of these demonstrated enlarged kidneys, renal cortical infarcts and thinning, papillary necrosis, acute renal atrophy, retroperitoneal hematoma and ureteral infarction, which were confirmed by autopsy studies. Hypertension developed in 7 patients. Urinary tract infection was uncommon and no patient had a clinical history compatible with chronic or acute pyelonephritis. Contrary to usual opinion our compatible clearly showed evidence of frequent and widespread renal pathology in paroxysmal nocturnal hemoglobinuria most likely due to repeated microvascular thromboses similar to the venous trombosis involving other organs in this disorder. Since most of these patients present initially to urologists knowledge of this entity is mandatory.

Adolescent↗
PubMed1991

Paroxysmal nocturnal hemoglobinuria with onset in childhood and adolescence.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematologic disorder characterized by hemoglobinuria, thrombosis, infection, and a tendency toward bone marrow aplasia. Onset usually occurs in adulthood. Few children and adolescents with PNH have been described, and data on diagnosis, clinical course, and survival in young patients are unavailable. METHODS: We retrospectively reviewed clinical and laboratory data on all patients 21 years old or younger in whom PNH had been diagnosed at Duke University Medical Center from 1966 to 1991. RESULTS: Medical records and clinical follow-up data were available for 26 young patients. Although 50 percent of adult patients present with hemoglobinuria, only four of our patients (15 percent) presented with this feature. In contrast, 15 of our patients (58 percent) had moderate or severe bone marrow failure at presentation, as compared with about 25 percent of adults in cases from the literature; all 26 patients eventually had evidence of bone marrow dysfunction. Eight patients (31 percent) have died, with a median survival of 13.5 years since their initial symptoms. CONCLUSIONS: Children and adolescents with PNH have a greater prevalence of bone marrow failure than do adults with this disorder, and their morbidity and mortality are high. Bone marrow transplantation should be considered for selected young patients with PNH.

Adolescent↗
PubMed2004

General anesthesia for cesarean delivery in a patient with paroxysmal nocturnal hemoglobinuria and thrombocytopenia.

UNLABELLED: Pregnancy in patients with paroxysmal nocturnal hemoglobinuria (PNH) increases the risk of complement activation, hemolysis, and thrombosis. We describe the anesthetic management of a patient with PNH who underwent general anesthesia for cesarean delivery. Steroids, heparin, and blood products were administered early to minimize the likelihood of a hematologic emergency. IMPLICATIONS: Pregnancy in patients with paroxysmal nocturnal hemoglobinuria increases the risk of hemolysis, thrombosis, anemia, and thrombocytopenia. We describe the anesthetic management of a patient with paroxysmal nocturnal hemoglobinuria and thrombocytopenia who underwent general anesthesia for cesarean delivery.

Adult↗
PubMed1989

Clinical studies on canine dirofilarial hemoglobinuria: measured and calculated serum osmolalities and osmolar gap.

Serum osmolalities and osmolar gap were determined in 43 normal healthy beagles (control group) and 40 dogs with dirofilarial hemoglobinuria (hemoglobinuria group). In the control group, the measured and calculated serum osmolality levels were in the means of 296 +/- 5 (SD) mOsm/kg and 293 +/- 6 mOsm/kg respectively, showing an osmolar gap less than 10 mOsm/kg. In the hemoglobinuria group, the measured serum osmolality ranged from 272 to 370 mOsm/kg. A considerable number of dogs had normal serum osmolalities in spite of severe intravascular hemolysis, suggesting that the changes in serum osmolality would not be the direct cause of intravascular hemolysis. The measured serum osmolality (331 +/- 28 mOsm/kg) was significantly higher in 11 dogs which died after a surgical removal heartworms than in 29 dogs which recovered after the removal (302 +/- 17 mOsm/kg). The calculated serum osmolality level was 296 +/- 16 mOsm/kg in 24 recovered cases, and 304 +/- 22 mOsm/kg in 10 fatal cases. The osmolar gap stayed in the normal ranges of 5.4 +/- 5.9 mOsm/kg in recovered cases, but it attained a higher level of 22.7 +/- 8.9 mOsm/kg in fatal cases, suggesting poor prognosis in cases with large osmolar gaps. There were significant positive correlations between the measured serum osmolality and osmolar gap, serum sodium, potassium, BUN, GOT, GPT, creatinine, bilirubin and plasma hemoglobin values, as well as between the osmolar gap and serum potassium, BUN, GOT, GPT, creatinine and bilirubin values. The plasma hemoglobin concentration fell markedly without significant change in serum osmolality 20 hr after the heartworm removal.

Animals↗