Massive Hemolysis Research Articles

Severe haemolysis associated with hepatitis A and normal glucose-6-phosphate dehydrogenase status.

We report massive acute haemolysis associated with hepatitis A in a 36 year old Irish woman. Although mild anaemia is not uncommon during viral hepatitis, haemolysis of the severity observed in this patient has previously been reported only in patients with glucose-6-phosphate dehydrogenase deficiency.

Paroxysmal nocturnal haemoglobinuria with coexisting deficiency of the ninth component of complement: lack of massive haemolytic attack

A 47-year-old woman with paroxysmal nocturnal haemoglobinuria (PNH) was found to have an inherited deficiency in the ninth complement component (C9). In complement-sensitivity lysis tests, 80% of her erythrocytes were markedly complement-sensitive (PNH-III). Laser cytofluorimetry with a monoclonal antibody against decay-accelerating factor (DAF) revealed that 95% of her erythrocytes were DAF-negative. Surprisingly, she has suffered only mild haemolysis and has never experienced massive spontaneous haemolysis. Gross haemoglobinuria and jaundice occurred only after receiving postoperative transfusion of whole blood. In her serum, C9 was not detectable either by immunological or by functional assays. Both the Ham test and the sugar water test using normal human serum or plasma yielded marked haemolysis of the patient's erythrocytes. When the patient's serum or plasma was used, only a trace of lysis was detected. Addition of purified human C9 to her plasma fully restored haemolysis. These observations indicated that C9 may play a critical role in haemolytic attacks in patients with PNH and that characteristic haemolysis in PNH may be tempered by coexisting C9 deficiency.

Fatal clostridial pancreatitis following ERCP and percutaneous needle biopsy

A patient with a large necrotic pancreatic carcinoma underwent ERCP and percutaneous biopsy of the cancer and of a suspected hepatic metastasis. On the second day, she developed a massive hemolysis with a 50% drop of hemoglobin level and a rise of serum bilirubin level from 1.3 to 37.5 mg%. Gas was noted on followup CT scan of the pancreas. Clostridium perfringens was found by needle aspiration of the pancreas and on multiple blood cultures. Death resulted from sepsis on the fourth day. Since ERCP and percutaneous needle biopsy of the pancreas are being done with increasing frequency, and since this complication has not been previously described, this case report is presented.

Cisplatin-associated hemolytic-uremic syndrome. Successful treatment with a staphylococcal protein A column

Cisplatin-associated hemolytic-uremic syndrome (HUS), an under-reported form of HUS induced by chemotherapy, typically pursues a fulminant and lethal course. We report the cases of two patients with squamous cell carcinoma of the head and neck who developed massive hemolysis, profound thrombocytopenia, and dialysis-dependent renal failure after therapy with cisplatin. Plasma exchange was ineffective in both patients, but plasma perfusion with a staphylococcal protein A column produced a dramatic and permanent response in the second patient. These cases show the importance of considering HUS as a cause of renal failure in such patients who receive cisplatin-based chemotherapy, and support the role of staphylococcal protein A plasma perfusion as treatment for this condition.

Clostridium perfringens Septicemia with Massive Hemolysis

Massive hemolysis with acute renal failure occurred in a previously healthy 69-year-old patient as a complication of Clostridium perfringens septicemia secondary to gall bladder empyema. To our knowledge, this is one of the few patients with C. perfringens septicemia and massive intravascular hemolysis who survived the episode and regained a normal renal function.

Massive intravascular hemolysis associated with intravenous immunoglobulin in bone marrow transplant recipients.

We report two group B Rho(D)-positive bone marrow transplant recipients who had sudden onset of massive intravascular hemolysis following the use of intravenous immunoglobulin (i.v. Ig). The first case developed chills and hypotension followed by hemoglobinuria with the first infusion of i.v. Ig. More severe symptoms occurred when infusion was reattempted. The second case developed hemoglobinuria without other symptomatology. In both patients, direct and indirect antiglobulin tests became positive following the use of i.v. Ig. Sera and eluates demonstrated IgG anti-B. Samples of i.v. Ig received by both patients contained both anti-B and anti-A and were incompatible with patients' red blood cells. These serologic findings indicate acute hemolysis secondary to the presence of IgG anti-B in i.v. Ig preparations.

Predictive Markers for Clostridium perfringens Sepsis in High-Risk Newborns

<i>Sir</i>.—Warren et al¹suggest that massive hemolysis in the newborn with necrotizing enterocolitis (NEC) may be due to<i>Clostridium perfringens</i>infection. In our experience, by the time massive hemolysis is manifest, systemic spread of<i>C perfringens</i>, its toxin, or both will have occurred, and institution of appropriate antibiotic therapy will probably be too late. We have carried out a long-term study on the patterns of the development of the enteric microflora in preterm neonates at high risk for NEC. Our data suggest that early appearance of<i>C perfringens</i>in the feces of low-birth-weight, preterm infants may predict NEC.² Although C<i>perfringens</i>appears early in the full-term infant's stool (depending on feed composition),³colonization patterns in preterm infants differ,⁴and therefore recognition of<i>C perfringens</i>in the feces provides a valuable marker for contamination. Furthermore, timing of colonization with<i>C perfringens</i>during the first 2 weeks

Multiple hornet stings with features of Reye's syndrome

Hornet's venom is known to possess a variety of toxic effects. A 19-mo-old girl who developed a Reye-like syndrome following multiple stings by the Oriental hornet (Vespa orientalis) is described. She presented with encephalopathy associated with hepatomegaly, elevated transaminase levels, low prothrombin time, and hyperammonemia. Liver biopsy demonstrated microvesicular fatty infiltration and diffuse mitochondrial changes. Additional features were acute renal tubular necrosis and massive hemolysis.

Massive intravascular hemolysis without serum haptoglobin depletion

Hyperplasia of marrow histiocytes with extensive hemophagocytosis was found in a patient with cytomegalovirus infection. He experienced massive intravascular hemolysis, but, unexpectedly, no depression of the serum haptoglobin level was found by either single radial immunodiffusion or rate nephelometry. The unexpectedly high haptoglobin value may have been the result of "blockade" of the monocyte-macrophage system, with resultant failure to clear haptoglobin-hemoglobin complex rapidly from the circulation. Use of the techniques described for the indirect estimation of unbound serum haptoglobin alone should avoid confusing results in similar clinical circumstances.

Increase of F cells during acute hemolysis in glucose-6-phosphate dehydrogenase-deficient males.

Five male Sicilian children with glucose-6-phosphate dehydrogenase deficiency were studied shortly after hemolytic crisis in order to evaluate the immediate effects of massive hemolysis on fetal Hb (HbF) levels and the number of circulating F cells. Hematological values seen 4 months after the children recovered from the crisis were considered representative of the patients' steady state. All patients had an increase in HbF levels (2.26 +/- 0.24%) and F cell number (29 +/- 4.79%) in the acute phase and their HbF values and F cells returned to normal range at control. Globin synthesis was balanced in the peripheral blood and bone marrow and there was a small peak of gamma chains. Globin chain electrophoresis showed that both G gamma and A gamma genes were active in all patients. These results confirm that hemolytic stress produces increased F cell release in peripheral blood. Such release is rapid enough (less than 72 h) to be consistent with the hypothesis of an induction of HbF synthesis in late erythroid precursors.

HEMOLYTIC TRANSFUSION REACTION (HTR) ASSOCIATED WITH INTRAVENOUS IMMUNE GLOBULIN (IV Ig)

High dose IV Ig has been tried in immune cytopenia(s) with few complications. We report a severe HTR due to IV Ig. A 10 yr-old B Rho(D) pos. boy who received a bone marrow transplant (BMT) from his O Rho(D) neg. sister was given IV Ig (C51911, GAMIMUNE, 5%, Cutter Lab.) for platelet alloimmunization. On day 9 post-BMT he developed a chill and hypotension after 120 ml of IV Ig and hemoglobinuria later. On day 10 he had a severe shaking chill after 100 ml of IV Ig and symptoms recurred after 2 ml of infusion. On days 10 to 12 he had hyperbilirubinemia (4.5 mg/dl), hemoglobinemia (98 mg/dl), hemoglobinuria (75 mg/dl) and no haptoglobin. Hb remained between 6-7 g/dl with 8 units of RBC. On day 13 urine became clear and Hb rose without transfusions (Tx). No Tx were given 48 hrs. pre-IV Ig. After BMT he was given O neg. deglycerolized-RBC and group B plasma and platelets. Pre-IV Ig he was B pos. and had a negative direct antiglobulin test (AGT) and antibody screen. Post-IV Ig direct and indirect AGT were positive (days 10-13); anti-B (1:4) was detected in his sera from day 12. Sera and eluate demonstrated antiglobulin reactivity with group B RBC. The IV Ig he received had 1:64 for anti-B and Anti-A. On day 16, patient was typed as O neg. without reticulocytosis, indicating massive intravascular hemolysis of autologous RBC, not a response to BMT. Since blood group antibodies are present in human immune serum globulin preparations, compatibility test should be done when using IV Ig to prevent HTR.

Implication of Plasma Free Hemoglobin in Massive Clostridial Hemolysis

Prolonged tissue oxygenation and maintenance of intravascular oncotic pressure are severely impaired with the absence of an effective RBC mass. We recently encountered a patient with a nondetectable hematocrit value (0%) attributed to Clostridium perfringens hemolysis. The patient maintained normal BP, tissue oxygenation, and mentation and survived longer than four hours, despite ineffective transfusion therapy. Plasma free hemoglobin was responsible for the preservation of tissue oxygenation, intravascular oncotic pressure, and pH. Existing studies in animals support stroma-free hemoglobin as an effective blood product in humans.

Implication of plasma free hemoglobin in massive clostridial hemolysis

Implication of plasma free hemoglobin in massive clostridial hemolysis

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2'-Deoxycoformycin (dCF), a tight-binding inhibitor of adenosine deaminase, has recently been entered into clinical trials. Toxicity has included lymphopenia, seizures, coma, conjunctivitis, renal failure, and hemolysis. Mice treated with dCF on a variety of schedules exhibited massive hemolysis. Hemolysis was brief, lasting about 20 hours, and did not recur upon readministration of the drug unless readministration was delayed for at least 6 days after initial exposure, which suggests that a sensitive subpopulation of cells was selectively destroyed. Splenectomy failed to protect the animals from dCF-induced hemolysis. Administration of adenosine or 2'-deoxyadenosine without dCF did not cause hemolysis, and use of these two agents with dCF did not potentiate the observed hemolysis. ATP and dATP levels were measured in erythrocytes, and changes in levels of these nucleotides did not correspond with the development of hemolysis.

Accidental Systemic Exposure to Sodium Hypochlorite (Clorox) during Hemodialysis

A case of accidental exposure of a patient undergoing hemodialysis to a sodium hypochlorite solution is reported. A 61-year-old woman was completing a hemodialysis treatment when routine cleaning of the hemodialysis machine was started. Approximately two liters of undiluted sodium hypochlorite cleaning solution (Chlorox) was added to the dialysis bath, soaking the membrane fibers. For less than two minutes the Chlorox-soaked membrane was in contact with the blood returning to the patient. This accident led to massive hemolysis, hyperkalemia, cyanosis, and cardiopulmonary arrest. Hemolysis may have been caused by the hypertonic solution, rapid exothermic protein degradation, alkaline degradation, or another mechanism. The sudden rise and fall in the concentrations of serum electrolytes and subsequent hyperkalemia was the most probable cause for the cardiac arrest. Cardiopulmonary resuscitation was started, the patient was intubated, given oxygen, sodium bicarbonate, atropine, dopamine, and isoproterenol. Sodium thiosulfate 5 g was administered by a nasogastric tube approximately 25 minutes after the cardiac arrest as a neutralizing reducing agent. The patient's condition stabilized, and she recovered after a week of hospitalization. Cleaning solutions used in the routine cleaning of hemodialysis machinery represent potentially toxic agents. Hemodialysis procedures should ensure that cleaning and sterilizing solutions cannot accidentally come into contact with a dialysis machine that is still connected to the patient.

Toxic effects in mouse and rat of rubescenslysin from Amanita rubescens

Rubescenslysin, a protein derived from the edible fungus Amanita rubescens (Persoon ex Fries) Gray, has a direct cytolytic action. When given i.v. it is highly toxic: the ld 50 was 153 (119–197) haemolytic units (HU)/kg for rats and 312 (281–326) HU/kg for mice, equivalent to approximately 0·15 or 0·31 mg protein/kg, respectively. Poisoning was characterized by intravascular haemolysis, massive exudation of plasma with alveolar obstruction due to an increase in vascular permeability, cardiotoxicity and central nervous effects. Which of these phenomena predominated depended on the survival time, i.e. the dose and the rate of administration of the toxin. After large doses (600 HU/kg mouse) death ensued in as short a time as 10–15 sec and was accompanied by seizures. Doses in the region of the ld 50 or somewhat higher (300–400 HU/kg mouse or 200 HU/kg rat) killed mice in 30 sec to 60 min and rats in 7–15 min. Massive haemolysis and plasma exudation dominated the clinical picture and determined the biochemical, macroscopic and histological findings. Death resulted from noncardiac, haemorrhagic pulmonary oedema. The cardiac effects were relatively inconspicuous. Sublethal doses caused intravascular haemolysis of mild degree; haematuria being the most striking symptom.

Renal disease in acute Plasmodium falciparum infection in man

Falciparum malaria is a complex disease with a broad clinical spectrum. Several pathophysiologic changes are involved in the disease process. Yet, in the complicated chain of events, these pathophysiologic phenomena are not specific and may be found in other acute inflammatory diseases. One of the most important effects of Plasmodium falciparum infection is capillary endothelial damage, which causes increased vascular permeability leading to an impairment in the microcirculation. Hemodynamic alteration, hematologic change, and immunologic response are among the major pathophysiologic mechanisms in the pathogenesis of the disease. Based on these mechanisms, renal involvement in falciparum malaria therefore varies widely. Disturbances in the renal microcirculation are responsible for acute renal failure; massive intravascular hemolysis causes hemoglobinuria with or without renal failure; and immunologic reaction to parasites accounts for glomerular lesions. In addition, fluid and electrolyte disorders may result from the nonspecific effect of fever. In this paper, the clinical spectrum of renal manifestations, renal pathologic changes, and pathogenesis will be presented.

The effect of copper excess on iron metabolism in sheep.

Sheep were treated with large amounts of copper (20 mg of CuSO4,5H2O/kg body wt. per day) for 9 weeks to examine the effect of copper excess on iron metabolism. In addition to confirming that massive haemolysis and accumulation of copper occurs in the liver, kidney and plasma after 7 weeks of exposure to excess copper, it was observed that excess copper produced an increased plasma iron concentration and transferrin saturation within 1 week. Further, iron preferentially accumulated in the spleen between 4 and 6 weeks of copper treatment, producing 3-fold increases in the iron content of both the ferritin and non-ferritin fractions. A 3-4 fold increase was also observed in the amount of ferritin that could be isolated from the spleen. The copper treatment had little or no effect on the concentration of iron in the liver and bone marrow. The following properties of erythrocytes were also unaffected by copper treatment: size, haemoglobin content and pyruvate kinase activity, although the erythrocyte concentration of copper increased after 6 weeks. Copper accumulated in the spleen between 6 and 9 weeks, probably owing to the phagocytosis of erythrocytes containing high concentrations of copper. The data suggest that copper excess influences iron metabolism, initially by causing a compensated haemolytic anaemia, and later by interfering with re-utilization of iron from ferritin in the reticuloendothelial cells of the spleen.

Acute Babesia bigemina infection: Changes in coagulation and kallikrein parameters

Plasma from splenectomized calves infected with B. bigemina were assayed for disturbances in the coagulation system. Changes were observed in the WBCT, PTT, PT, TT and RT as well as in fibrinogen, calcium and thrombocyte levels. These changes were most marked during days 6--7 when parasite numbers were greatest. Massive intramuscular haemolysis coincided with these changes. The plasma kallikrein level fell progressively from days 6--11 and the level of activated kallikrein rose slightly. Parasite doubling time was calculated at 8.13 h. The similarities and differences between this disease and B. argentina infections of cattle are discussed.

Massive hemolysis caused by acetaminophen. Positive determination by direct Coombs test

Massive hemolysis caused by acetaminophen. Positive determination by direct Coombs test