Massive Intravascular Hemolysis Research Articles

Fatal Disseminated Intravascular Coagulation and Autoimmune Hemolytic Anemia Due to Anti-Ena

Background: Autoimmune hemolytic anemia (AIHA) caused by warm IgG antibodies is usually characterized by IgG-mediated extravascular hemolysis and anemia that develops slowly. We report a case of AIHA presenting with sudden massive intravascular hemolysis and disseminated intravascular coagulation (DIC), similar to that seen following an ABO incompatible transfusion reaction.Case Report: A 71-year-old female had a 4-year history of immune thrombocytopenic purpura requiring splenectomy 1 ½ years ago. Six weeks prior to admission, in association with a viral infection, her platelet (plt) count fell from 350,000/mm3 to 150,000/mm3 over 2 weeks. She was treated with oral prednisone, and one week prior to admission her platelet count was 200,000/mm3. Two days before admission she noted reddish urine. Laboratory studies at her doctor's office showed hemoglobin (Hb) 9 g/dL (had been 13 g/dL 1 week earlier) and bilirubin (bili) 9 mg/dL. On the day of admission, in the emergency department, she complained of generalized weakness, jaundice, and near-syncope. She became increasingly agitated and dyspneic and was intubated. At that time laboratory studies showed Hb 4.5 g/dL, LDH >2,500 U/L, haptoglobin 7 mg/dL, total bili 13.0 mg/dL, indirect bili 11.8 mg/dL, D-dimers >10,000 ng/dL, plts 139,000/mm3, prothrombin time 18.3 seconds, fibrinogen 166 mg/dL, fibrin monomers were positive. She was admitted to the medical intensive care unit with a diagnosis of DIC and hemolysis. The patient's RBCs were coated with IgG (4+) and C3 (3+); IgM and IgA were not detected. Her plasma and an eluate contained an antiglobulin test reactive autoantibody with anti-Ena specificity (anti-Pr and -Wrb were excluded), an antibody directed at epitopes on glycophorin A. No alloantibodies were detected in the patient's plasma. The patient was treated with IV steroids and received multiple RBC transfusions over the first few hospital days. Her Hb stabilized at 10 g/dL. The day after admission, she became oliguric and required dialysis throughout her hospital course. Over the next several days, additional signs and symptoms of DIC were noted: ischemic digits and toes, elevated Troponin I, partial thromboplastin time, D-dimers and decreased plt count. She was placed on intravenous heparin. Gastrointestinal bleeding developed and heparin was discontinued on hospital day 9. One week later, the onset of abdominal pain necessitated exploratory laparotomy which revealed ischemic bowel with perforation; vessels showed organized thrombi with recanalization. She remained critically ill and died on hospital day 25.Conclusion: Complement-mediated intravascular hemolysis in a patient with AIHA due to anti-Ena was associated with renal failure and DIC leading to death of the patient. All of these, even separately, are unusual in warm AIHA. We know of no reports where all 5 events (intravascular lysis, anti-Ena, renal failure, DIC, and death) occurred in one patient.

Recurrent acute renal failure complicating IgG warm-type autoimmune intravascular haemolysis

A 44-year-old woman experienced recurrent episodes of massive intravascular haemolysis complicated by acute oliguric renal failure over a period of 22 years. The haemolysis was induced by IgG warm type autoantibody and complement and responded to corticosteroid therapy. The renal failure was treated effectively by dialysis. To our knowledge, such a life long occurrence of recurrent intravascular haemolysis induced by IgG warm type autoantibodies together with renal failure has not been reported before.

Intravascular Hemolysis and Acute Renal Failure After Mitral and Aortic Valve Repair

Intravascular Hemolysis and Acute Renal Failure After Mitral and Aortic Valve Repair

Intravascular Hemolysis of Red Blood Cells in the Development of Organ Dysfunctions in Critical Conditions

The review analyzes the literature data, which suggest that microcirculatory bed disorders underlie organ dysfunctions in critical conditions, are largely associated with intravascular hemolysis of red blood cells, release of free hemoglobin and its subsequent catabolism to Fe2+ ions. Failure in the coagulation system, transport, and utilization of the trace element is assumed to develop during massive intravascular hemolysis. Excess Fe2+ ions that have a high catalytic activity may exert a direct damaging effect on the capillary endothelium with the development of capillary leakage. The large number of Fe2+ ions under hypoxia, acidosis, and reperfusion eliminate the major substrate for endogenous antioxidants — hydrogen peroxide, as well as superoxide radical, from the reactions of neutralization of primary active radicals, which is favorable to the activation of lipid peroxidation processes and the synthesis of more toxic secondary radicals.

Massive Intravascular Hemolysis and a Rapidly Fatal Outcome

Massive Intravascular Hemolysis and a Rapidly Fatal Outcome

Impact of harvest product volume in erythrocyte depletion of allogeneic or autologous bone marrow using COBE spectra

Depletion of bone marrow (BM) from erythrocytes is used to prevent early hemolysis in major ABO incompatible allogeneic hematopoietic cell transplantation (Allo-HCT). This method was also strongly recommended before storing of autologous and even allo-BM for volume reduction in order to prevent early hemolysis and DMSO toxicity after infusion. In our center, erythrocyte depletion of BM harvests has been performed on a continuous flow cell separator, which used a closed system with a high mononuclear cell (MNC) yield and low rate of erythrocyte contamination. According to the protocol of a cellular therapy approach in a cardiovascular collaborative study we have to adopt the process to lower volumes. We aimed to compare our results with standard volume (SV) (historical control) to low volume ED procedures. Patients and method Data has been collected from the last five years. We analyzed 28 cases in the SV group (BM volume >750 ml) and 39 cases in the low volume (LV) group. Nineteen of these cases were allogeneic, and 48 were autologous procedures. We used the software COBE PBSC coll vers 5.1 and a standard disposable set (Gambro BCT, Lakewood, USA) for the procedure, and simultaneously, a double bag system with intermediate connectors were used to overcome re-circulation (COBE Spectra Bone Marrow Processing Set, Lakewood USA). Results The mean volume reduction was 88% (range, 84.4–93.5%) for SV and 90.8% (range, 87.2–91.3%) for the LV group. We did not find any significant difference for MNC yield, volume reduction rate and CD34+ cell recovery between the SV and LV group. There were no complications experienced with regards to device or technical difficulties during procedures. Acute massive intravascular hemolysis was not observed in allogeneic recipients. Conclusion ED and volume reduction with COBE spectra produced successful results in standard and low harvest volumes. This process can be successfully applied to lower volumes and comparable results to the SV harvest can be achieved for the ED rate, reduction of volume and recovery of MNCs and CD34+ cells.

A Case of Massive Intravascular Hemolysis Associated with Clostiridium perfringens Sepsis

Clostridium perfringens is an anaerobic, gram-positive rod that inhabits the soil and the intestinal tracts of many animals, including humans. C. perfringens is a major cause of food poisoning, traumatic or nontraumatic myonecrosis, clostridial cellulitis, gangrenous cholecystitis, sepsis or bacteremia, and intra- vascular hemolysis. Massive intravascular hemolysis is a rare complication of C. perfringens septicemia and has a high mortality rate with an extremely rapid progression. Therefore, aggressive treatment is required as soon as the diagnosis is made. In this study, we re port a case of massive intravascular hemol- ysis due to C. perfringens septicemia in a 34-year-old man with liver cirrhosis. (Korean J Hematol 2007;

Massive intravascular hemolysis in a patient infected by a Clostridium perfringens.

Clostridium perfringens infection is a very rare cause of massive intravascular hemolysis, but it should always be kept in mind, since only early treatment can rescue patients from an otherwise rapidly fatal outcome. We report a case of a 78-year-old diabetic male who was admitted complaining of general fatigue, dark red urine, and vomiting. His blood revealed massive hemolysis. Computer tomography demonstrated huge liver abscess in the right lobe of the liver. About 1 h after admission, he suddenly fell into a critical condition. He died 3 h after admission in spite of intensive care and resuscitation. Clostridium perfringens was detected from the blood taken before death and from liver abscess by biopsy after death. We concluded that this patient died of acute massive intravascular hemolysis in septicemia caused by Clostridium perfringens infection.

A Case of Clostridium perfringens Septicemia with Fatal Hemolytic Complication

Massive intravascular hemolysis secondary to Clostridium perfringens septicemia is rare but often fatal. We report a case of a fatal clostridial hemolytic complication in a 71-year-old woman with probable refractory anemia. The patient was admitted to the emergency room due to a comatose mental state and a high fever. Laboratory analysis showed massive hemolysis. She died from severe anemia two hours after admission. The next day, blood cultures grew gram positive cocci and boxcarshaped gram positive rods, which were identified as coagulase-negative staphylococci and C. perfringens, respectively.

Massive intravascular haemolysis with T‐activation and disseminated intravascular coagulation due to clostridial sepsis

An 80-year-old man presented to the emergency department with acute onset of rigors, dyspnoea and delirium, after 2 d of abdominal pain. He had a past history of cholecystectomy and type II diabetes mellitus. On arrival he was febrile and jaundiced with a tender abdomen. Spun blood samples revealed bright red plasma consistent with intravascular haemolysis (top left). The total haemoglobin was 8.7 g/dl, of which 6.7 g/dl was free plasma haemoglobin. The blood film demonstrated severe anaemia with microspherocytes and red cell fragments, toxic vacuolation, phagocytosed bacilli and marked thrombocytopenia (top right). Coagulation studies were consistent with disseminated intravascular coagulation (DIC). The results of a lectin panel were consistent with red cell T-activation. The source of sepsis was demonstrated by an abdominal computed tomography scan, which showed a gascontaining liver abscess with pneumobilia (bottom). Together these findings were characteristic of clostridial sepsis with massive intravascular haemolysis. In this case, despite recognition of the diagnosis on presentation, the patient did not respond to aggressive directed therapy and died within 3 h of admission. Blood cultures later revealed Clostridium perfringens. In adults, clostridial sepsis typically occurs in patients with gastrointestinal or genitourinary abscesses or following recent abdominal surgery. The patients often have an underlying malignancy or diabetes mellitus. Although infrequent, massive intravascular haemolysis is a classic complication of clostridial sepsis. It has a high mortality rate, despite prompt treatment with appropriate antibiotic and supportive therapy. Intravascular haemolysis results from the enzymes produced by Clostridia species. Alpha-lecithinase directly causes red cell membrane disruption and is likely to be the most significant cause of haemolysis. Neuraminidase acts on red cell membranes, cleaving off N-acetyl-neuraminic acid to expose the normally hidden T-cryptantigen (T-activation). Exposed T-antigen is then the target of anti-T, a naturally occurring haemolytic antibody present in all adult plasma. However, the contribution of T-activation to haemolysis is likely to be minor. In addition to the effects of clostridial enzymes, microangiopathy from associated DIC may contribute to intravascular haemolysis.

Babesiosis masquerading as recurrent immune thrombocytopenic purpura in a splenectomized patient

Dear Editor, Splenectomy is a clinically effective therapeutic intervention against chronic immune thrombocytopenic purpura (ITP) [1]. Severe, potentially life-threatening infections are a major long-term complication of splenectomy [2]. Certain infections may present with thrombocytopenia, which, in the case of splenectomized patients with a history of ITP, may be mistaken for ITP recurrence. We report what is, to the best of our knowledge, the first case of babesiosis in a patient with stable chronic ITP who had previously undergone splenectomy. The patient was a 66-year-old woman from Nantucket Island, MA, USA, with a known diagnosis of ITP, who was treated with prednisone, intravenous immunoglobulin, and rituximab before eventually undergoing splenectomy. Her postsplenectomy platelet count remained stable in the 60,000–70,000 range, without treatment. Two years following splenectomy, she presented with fever (temperature 101°F), chills, myalgias, and arthralgias. She denied any bleeding or bruising. Physical examination was unremarkable. Complete blood count demonstrated a hematocrit of 45.5 vol%, a white blood cell count of 4,600 cells/μL, and a platelet count of 10,000 platelets/μL. Liver function tests were notable for a slightly elevated alanine transaminase of 64 and aspartate transaminase of 49. Peripheral smear showed characteristic Howell-Jolly bodies and rare intraerythrocytic parasite ring forms in normal-sized red blood cells (Fig. 1). Babesiosis was confirmed by extremely high IgG and IgM titers to Babesia of 1:1,024 and 1:1,280, respectively, and a positive polymerase chain reaction (PCR). Importantly, there were no detectable antibodies for Lyme disease and ehrlichiosis. The patient was started on azithromycin and atovaquone and on a tapering dose of prednisone. She received 4 weeks of antibiotic therapy and her platelet count increased to 325,000 platelets/μL. Four weeks after completing the course of antibiotics, the platelet count dropped again to 24,000 platelets/μL. She continued to have circulating intracellular parasites on peripheral smear, and Babesia PCR and serologies were still positive. Atovaquone, azithromycin, and prednisone treatments were restarted. Her platelet count steadily improved, and only after 4 months of antibiotic therapy did the PCR for Babesia normalize. Steroids were discontinued. Her platelet count returned to her baseline value of 60,000–70,000, off immunosuppressive and anti-infective therapies. The approach to thrombocytopenia should always include an accurate history, physical examination, and examination of the peripheral blood smear. Splenectomy predisposes to infections that may present with thrombocytopenia, including ehrlichiosis, Colorado tick fever, Rocky Mountain spotted fever, babesiosis, and malaria. While patients can have a recurrence of ITP, other causes of thrombocytopenia need to be excluded. Babesiosis is a worldwide tick-borne disease caused by the intraerythrocytic protozoan Babesia [3]. Humans are infected by various tick vectors (Ixodes scapularis and Ixodes ricinus) and rarely by transfusions. In the USA, endemic areas include the islands off the coasts of MA and NY [4]. Symptoms include fever, chills, weakness, and myalgias. Babesiosis may present with hemolytic anemia, lymphopenia, and thrombocytopenia. Splenectomized patients may develop a fulminant infection with massive intravascular hemolysis [5]. The peripheral blood smear reveals small coccoid and dumbbell-shaped organisms. The trophozoites are small rings similar to those of Plasmodium falciparum. The parasites can be pyriform (pear shaped). Babesia microti is associated with Maltese cross forms and ring forms. IgM or P. A. Konstantinopoulos . K. B. Miller . B. J. Dezube (*) Division of Hematology/Oncology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA e-mail: bdezube@bidmc.harvard.edu Tel.: +1-617-6677082 Fax: +1-617-9758030

Fatal septicemia due to Clostridium hathewayi and Campylobacter hominis

Clostridium hathewayi and Campylobacter hominis have not been previously reported in infection. We report a fatal case of septicemia, massive intravascular hemolysis, shock, and disseminated intravascular coagulation; both of these organisms were recovered on blood culture, although it seems likely that the C. hathewayi was responsible for the clinical picture and that the C. hominis was an incidental finding.

Massive Intravascular Hemolysis: A Fatal Complication of Clostridium Perfringens Septicemia in a Patient with Acute Myeloid Leukemia

We describe a fatal case of a 74-year old man with acute myeloid leukemia complicated by massive intravascular hemolysis due to Clostridium perfringens sepsis.

Clostridium perfringens-induced massive intravascular hemolysis complicating the course of colonic carcinoma

Introduction The incidence of Clostridium perfringens bacteremia in the general hospital population is low, ranging fkom 0.3 to 2.6%. The clinical spectrum of disease varies from a positive blood culture in an otherwise asymptomatic patient to fulminant sepsis characterized by fever, hy-potension, and, in some cases, massive intravascular hemolysis. The overall prognosis in this population is poor, especially when acute hemolytic anemia, gas gangrene, or diffise spreading cellulitis supervenes. We describe a case of fatal C. perfiingens bacteremia complicating the course of a 47-year-old male with a perforated colonic adenocarcinoma. The most common malignancies complicated by C. perfringens are leukemias and solid tumors of the genitourinary and gastrointestinal tracts. We comment on interesting clinicopathologic aspects of the case, including the association with malignancy, rapidly developing massive intravascular hemolysis, and diagnostic considerations.

Tubular injury as a cardinal pathologic feature in human heme oxygenase-1 deficiency

Heme oxygenase (HO) catalyzes degradation of heme to biliverdin, iron, and carbon monoxide. It consists of three isoforms: an inducible form (HO-1), a constitutive form (HO-2), and the third isoform (HO-3), with properties similar to HO-2. There is limited evidence to suggest that the induction of HO-1 may have anti-inflammatory effects in an in vivo model of oxidative stress–mediated renal injury. We experienced the first human case of HO-1 deficiency. The patient had persistent proteinuria and hematuria, with biochemical evidence of renal tubular injury. We obtained three consecutive renal specimens: two from renal biopsies at 2 and 5 years of age and the third from autopsy at 6 years of age. The patient had systemic vascular endothelial-cell injury with massive intravascular hemolysis. The serum was loaded with heme and a large amount of heme-conjugated haptoglobin. A high concentration of haptoglobin was also detectable in urine. Mesangial proliferation or change in glomerular capillary-wall thickness was relatively mild to moderate in all specimens. Electron microscopic examination showed widespread endothelial detachment and subendothelial deposits of an unidentifiable material. It was striking that tubulointerstitial injury, with tubular dilatation and/or atrophy, interstitial fibrosis, and inflammatory cell infiltration, advanced progressively. Tubular epithelial cells were injured, and massive deposition of iron and haptoglobin was detectable. Bowman's capsules were dilated significantly, probably secondary to the collapse of atrophic tubuli. This is the first report to show that HO-1 has critical roles in vivo in protecting renal tubuli, in addition to vascular endothelium, from oxidative injury.

Clostridium perfringens septicemia with massive hemolysis in a patient with Hodgkin's lymphoma

A 55-year-old woman with stage IV-B nodular sclerosing Hodgkin's lymphoma presented to the emergency department with fever and lethargy of 12 hours' duration. The patient developed massive intravascular hemolysis secondary to Clostridium perfringens sepsis and cardiac arrest unresponsive to transfusions and cardiac pulmonary resuscitation, and died within 4 hours of presentation. The differential diagnosis of massive intravascular hemolysis, as well as the pathogenesis and treatment of C perfringens-induced hemolysis, are discussed.

Haemolysis complicating acute viral hepatitis in patients with normal or deficient glucose-6-phosphate dehydrogenase activity.

Haemolytic anaemia as a complication of acute hepatitis has been reported in up to 23% of patients. However, the incidence may rise up to 70-87% in patients who have glucose-6-phosphate dehydrogenase (G6PD) deficiency. Massive intravascular haemolysis with renal failure, hepatic encephalopathy and even death have been reported. In our retrospective study of patients with acute viral hepatitis, the overall incidence of acute haemolysis was 4% (17/434). Only 53% (9/17) of them had G6PD deficiency. Patients with acute haemolysis had a significantly higher peak bilirubin level and required more prolonged hospitalization. Since hepatitis A virus vaccination, unlike hepatitis B virus vaccination, is not yet recommended for routine immunization, we suggest subjects who are G6PD-deficient should be vaccinated against hepatitis A. In endemic areas of hepatitis A virus infection, universal immunization remains the definitive answer.

Abdominal Pain and Massive Intravascular Hemolysis in a 47-Year-Old Man

One week prior to admission to the hospital, a previously healthy 47-year-old man was seen by a personal physician for epigastric pain. He was treated with acetaminophen and an antacid, which resulted in mild improvement. Over the next few days, however, he developed chills and rigors. On the morning of admission, he was difficult to arouse and was therefore brought to the emergency department.

Acute intravascular haemolysis following exchange transfusion with G-6-PD deficient blood

A neonate with hyperbilirubinaemia who developed massive intravascular haemolysis following exchange transfusion with glucose-6-phosphate dehydrogenase deficient blood is described. It is recommended that in areas endemic for this enzyme deficiency the donor blood should be screened before being used for exchange transfusion.

Investigation of the pathogenesis of massive hemolysis in a case ofClostridium perfringens septicemia

Massive hemolysis is a rare, usually fatal complication of Clostridium perfringens septicemia. Of all toxins produced by the bacterium, phospholipase C (PLC) is believed to be the most likely cause of hemolysis. An influence of neuraminidase has often been suspected. In the present study, a case of C. perfringens septicemia with acute massive intravascular hemolysis is described. It led to death within 4 h of admission to the hospital. While the course of events was comparable to previously reported cases, we succeeded in gaining deeper insight into the pathogenesis by monitoring serum anti-T titer and quantifying serum PLC activity during the course of the disease. We excluded an effect of neuraminidase by a negative direct antiglobulin test, a negative anti-T lectin test, and a steady serum anti-T titer of 1 in 32. Serum PLC activity, on the other hand, showed a nearly fivefold increase (6.0 to 27.3 U/l), which is consistent with the hypothesized dominant role of this enzyme.