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1.  Warm antibody autoimmune haemolytic anaemia associated with ovarian teratoma 
BMJ Case Reports  2010;2010:bcr06.2009.1971.
The ovarian cystic teratoma is a rare cause of autoimmune haemolytic anaemia by warm antibodies, resistant to corticotherapy, with few case reports published in the medical literature.
We present a case of a 45-year-old woman admitted to hospital due to general weakness. Laboratory studies revealed macrocytic anaemia, biochemical parameters of haemolysis and peripheral spherocytosis. The direct Coombs test was positive. Viral serologies, anti-nuclear antibodies, anti-double-stranded DNA antibodies and β2-microglobulin were negative. CT scan of the thorax, abdomen and pelvis showed a heterogeneous right anexial lesion. The patient was treated with corticotherapy without improvement of anaemia. Regression of extra-vascular haemolysis and normalisation of haemoglobin was obtained only after laparoscopic splenectomy and right ooforectomy, and the histopathology of the right anexial mass revealed a cystic teratoma.
Previously published cases controlled the haemolysis by surgically removing the lesion associated with splenectomy.
PMCID: PMC3052837  PMID: 22750920
2.  Fatal cold agglutinin-induced haemolytic anaemia: a case report 
Cold agglutinin disease usually develops as a result of the production of a specific immunoglobulin M auto-antibody directed against the I/i and H antigens, precursors of the ABH and Lewis blood group substances, on red blood cells. Autoimmune and lymphoproliferative disorders, Mycoplasma pneumoniae and other infections can be associated with the production of cold agglutinins. In its classic presentation with haemolytic anaemia and Raynaud's syndrome, cold agglutinin disease is usually idiopathic. Several factors play a role in determining the ability of a cold agglutinin to induce a haemolytic anaemia such as antibody concentration and temperature range, in particular the highest temperature at which antibodies interact with red blood cells.
Case presentation
A 48-year-old Caucasian man presented to our hospital with symptoms of extreme asthenia caused by severe anaemia. The transfusion of red blood cells (O Rh-positive), started as prescribed by the emergency guidelines in force without pre-transfusion tests, induced fatal haemolysis because of the presence of high levels of anti-H antibodies in his blood, that reacted with the large amount of H antigen in universal (0) red blood cells.
Emergency transfusion of universal red blood cells (0 Rh-positive or negative) is usually accepted by the international guidelines in force in emergency departments. In this report we describe a rare complication caused by the very high concentration in the recipient of cold agglutinins and the activation of the complement system, responsible for red blood cell lysis and consequent fatal cardiovascular shock. We conclude that emergency transfusion of universal red blood cells (0 Rh-positive or negative) may be dangerous and its risk should be assessed against the risk of delaying transfusion until the pre-transfusion tests are completed.
PMCID: PMC2923177  PMID: 20691050
3.  Severe delayed autoimmune haemolytic anaemia following artesunate administration in severe malaria: a case report 
Malaria Journal  2014;13(1):398.
Parenteral artesunate is recommended as first-line therapy for severe and complicated malaria. Although its efficacy has been proven, long-term safety profile is still under evaluation. Several cases of delayed haemolytic anaemia occurred after initial clinical improvement and resolution of parasitaemia in non-immune travellers and children living in endemic areas. Reports have generated concern that this phenomenon might be related to the treatment itself, either by direct toxicity or immune-related mechanism. This is a report of the first case of autoimmune haemolytic anaemia following treatment of severe malaria initially managed with parenteral artesunate with strong indication for drug-immune related mechanism.
A 17-year old Ivoirian female travelling in France presented with fever, headache and abdominal pain seven days after her arrival. Physical examination was indicative of septic shock while blood analysis showed normal haemoglobin level, but profound thrombocytopaenia and hyperlactataemia. Blood smear analysis showed Plasmodium falciparum infection with a parasitaemia of 0.8%. Severe malaria was diagnosed according to the WHO criteria. The patient was initially managed with artemether/lumefantrine combination and then parenteral artesunate for 48 hours. Empiric antibiotic course was also initiated with ceftriaxone, metronidazole, gentamycin, and then piperacillin and ciprofloxacin. At day 14, haemoglobin dropped to 4.6 g/dL with biologic features indicative of haemolysis (LDH 658 U/L, haptoglobin <0.15 g/L). At that time, parasitaemia was negative and other infections or hereditary disorders were excluded, while Coombs’ direct antiglobulin test was positive for IgG and C3d. Antinuclear antibodies were absent. Further investigations evidenced drug-induced antibodies related to artesunate. It was concluded a drug-mediated autoimmune haemolytic anaemia. A corticosteroids regimen was initiated at 1 mg/kg/day. Outcome was favourable and corticosteroids were progressively tapered during two months. At present the patient’s condition remains stable without recurrence of haemolytic anaemia.
This is the first case of delayed haemolytic anaemia related to artesunate with a strong indication for drug-immune related mechanism. Further research is warranted to better characterize this plausible cause of post-treatment haemolysis following parenteral artesunate administration in severe malaria patients.
Electronic supplementary material
The online version of this article (doi:10.1186/1475-2875-13-398) contains supplementary material, which is available to authorized users.
PMCID: PMC4203878  PMID: 25306236
Severe malaria; Artesunate; Autoimmune haemolytic anaemia; Side effects
4.  Haemolytic anaemia due to stenosed double-reinforced grafts after surgical repaired aortic dissection 
Haemolytic anaemia due to a stenosed graft is a rare complication after surgery for aortic dissection. We present the case of a patient with haemolytic anaemia and heart failure, who had undergone emergent ascending aorta replacement for type A acute aortic dissection 5 years earlier. Chest computed tomography revealed severe graft stenosis of the proximal anastomosis and transthoracic echocardiography showed severe aortic regurgitation. Surgical treatment was necessary because of heart failure and myocardial ischaemia due to haemolytic anaemia and aortic regurgitation. During the operation, we found an inner graft surrounded by an outer graft and a dilated lumen between the double-reinforced grafts compressing the inner graft. We successfully reconstructed the aortic root with a total arch replacement. To the best of our knowledge, there are no cases in which haemolytic anaemia and AR developed in a patient with acute aortic dissection surgically treated by such a mechanism.
PMCID: PMC3422964  PMID: 22695513
Haemolytic anaemia; Surgical repaired aortic dissection and aortic regurgitation
5.  Severe haemolytic anaemia after replacement of the mitral valve by a St Jude medical prosthesis. 
British Heart Journal  1989;62(6):475-476.
Severe haemolytic anaemia developed in a 33 year old patient after the mitral valve was replaced with a St Jude medical prosthesis. This was the patient's third thoracotomy. She had already had a mitral commissurotomy and a mitral valve bioprosthesis. The patient had an E+ antibody to red blood cells as well as a paraprosthetic leak. The haemolysis became less severe once the population of E+ red cells was completely haemolysed. However, the patient continued to require transfusions to remain out of heart failure. Once the valve was replaced, the haemolysis subsided. Fulminant haemolysis after mitral valve replacement is rare. When it does occur, a paraprosthetic leak should be suspected. Other causes of haemolysis, however, must also be considered and these may contribute to the severity of haemolysis.
PMCID: PMC1216792  PMID: 2605063
6.  Mechanical haemolytic anaemia after valve repair operations for non-rheumatic mitral regurgitation. 
British Heart Journal  1980;44(4):381-385.
Two cases are described in which severe mechanical haemolytic anaemia developed shortly after operation for repair of non-rheumatic mitral regurgitation. One patient had a "floppy" valve and the other cleft mitral leaflets, and both had chordal rupture. In both there was residual regurgitation after repair though in one this was initially only trivial. Clinically manifest haemolysis ceased after replacement of the valve by a frame-mounted xenograft. There are two previously reported cases in which haemolytic anaemia followed an unsuccessful mitral valve repair operation. Subclinical haemolysis or mild haemolytic anaemia may occur with unoperated valve lesions, but hitherto frank haemolytic anaemia has been observed only when turbulent blood flow is associated with the presence of a prosthetic valve or patch of prosthetic fabric. In these four cases, however, polyester or Teflon sutures were the only foreign material, and it is suggested that when these are used for the repair of leaflets, particularly in non-rheumatic mitral valve disease, they may increase the damaging effect of turbulence on circulating red blood cells.
PMCID: PMC482414  PMID: 7426198
7.  Thrombotic Thrombocytopenic Purpura-Haemolytic Uremic Syndrome and pregnancy 
Thrombotic Thrombocytopenic Purpura-Haemolytic Uremic Syndrome (TTP-HUS) is a rare pregnancy and postpartum complication that may simulate the more common obstetric complications, preeclampsia and the syndrome of haemolysis, elevated liver functions tests, low platelets (HELLP). We describe a 26 years old patient who presented with peri-partum TTP-HUSand was initially treated as a case of HELLP syndrome without any improvement. A brief review of the current TTP-HUS treatment options in pregnancy is also presented.
PMCID: PMC4189868  PMID: 25309655
Thrombotic Thrombocytopenic Purpura-Haemolytic Uremic Syndrome; pregnancy
8.  Haemolytic anaemia in myelomatosis 
Postgraduate Medical Journal  1973;49(570):279-281.
A case of IgA myelomatosis with a haemolytic anaemia is described. No auto-antibodies could be found and the mechanism of the haemolysis was obscure.
Haemolytic anaemia is a rare complication but a search of the literature has revealed a few cases with a comparable shortening of red cell life-span, most without auto-antibodies but some with a positive Coombs' test.
PMCID: PMC2495690  PMID: 4760726
9.  Atypical Haemolytic Uraemic Syndrome Associated with a Hybrid Complement Gene 
PLoS Medicine  2006;3(10):e431.
Sequence analysis of the regulators of complement activation (RCA) cluster of genes at chromosome position 1q32 shows evidence of several large genomic duplications. These duplications have resulted in a high degree of sequence identity between the gene for factor H (CFH) and the genes for the five factor H-related proteins (CFHL1–5; aliases CFHR1–5). CFH mutations have been described in association with atypical haemolytic uraemic syndrome (aHUS). The majority of the mutations are missense changes that cluster in the C-terminal region and impair the ability of factor H to regulate surface-bound C3b. Some have arisen as a result of gene conversion between CFH and CFHL1. In this study we tested the hypothesis that nonallelic homologous recombination between low-copy repeats in the RCA cluster could result in the formation of a hybrid CFH/CFHL1 gene that predisposes to the development of aHUS.
Methods and Findings
In a family with many cases of aHUS that segregate with the RCA cluster we used cDNA analysis, gene sequencing, and Southern blotting to show that affected individuals carry a heterozygous CFH/CFHL1 hybrid gene in which exons 1–21 are derived from CFH and exons 22/23 from CFHL1. This hybrid encodes a protein product identical to a functionally significant CFH mutant (c.3572C>T, S1191L and c.3590T>C, V1197A) that has been previously described in association with aHUS.
CFH mutation screening is recommended in all aHUS patients prior to renal transplantation because of the high risk of disease recurrence post-transplant in those known to have a CFH mutation. Because of our finding it will be necessary to implement additional screening strategies that will detect a hybrid CFH/CFHL1 gene.
Tim Goodship and colleagues have identified a heterozygousCFH/CFHL1 hybrid gene which encodes a protein product identical to one previously described in association with atypical hemolytic uremic syndrome.
Editors' Summary
Atypical hemolytic uremic (aHUS) syndrome is a rare, chronic disease that can run in families. People with the condition are prone to developing kidney failure and high blood pressure, and are likely to have a shorter life span than healthy people. Previous work done by a group of researchers in Newcastle-on-Tyne, UK looked at the genetic underpinnings of aHUS in three families suffering from the condition. They found a region of the genome that was linked with the disease in all three families. That region was known to contain a gene for a protein called “factor H,” as well as a number of other genes for proteins that are involved in the same pathway as factor H in controlling an ancient defence system called complement. This system helps antibodies to kill invaders by marking any cell that is not protected by proteins such as factor H. Our own cells would be under constant threat without protective proteins such as factor H. Later studies found simple genetic mutations in people with aHUS, in the genes coding for factor H. However, other work suggested that in some families with aHUS, simple genetic mutations might not be the cause; instead more complicated rearrangements of the genome might occur which would then result in an abnormal factor H that incorporated part of the gene for another protective protein called factor H related protein 1.
Why Was This Study Done?
The researchers knew that it was important to understand the exact genetic mutations linked with aHUS in different families. This was because the exact type of mutation would help them predict whether a kidney transplant is likely to be successful in treating an individual with aHUS who has developed kidney failure. In people with mutations affecting proteins produced by the kidney, a kidney transplant would be likely to work; but in people with mutations affecting factor H, which is produced by the liver, the disease would probably recur after a kidney transplant.
What Did the Researchers Do and Find?
In this study, the researchers went back to one of the three families with aHUS they had previously studied. The researchers had shown before that in this family, the disease was linked with the genome region containing factor H, but no precise mutation in that region had been found. This time, the researchers screened the genome of the family members and looked in particular for a specific rearrangement of the genome that they suspected might be involved. They found that the genomes in this family had been shuffled in the factor H region, resulting in an abnormal version of factor H being produced.
What Do These Findings Mean?
The mutation these researchers identified is likely to result in development of aHUS that does not get better after a kidney transplant, because the abnormal factor H would still be produced in the liver after a transplant had been done. Therefore, the researchers suggest that patients with aHUS be checked for this particular mutation before it is decided whether to go ahead with a transplant.
Additional Information.
Please access these Web sites via the online version of this summary at
US National Institutes of Health Office of Rare Diseases information about atypical hemolytic uremic syndrome
The Online Mendelian Inheritance in Man (OMIM) contains an entry on hemolytic uremic syndrome. OMIM is a database of human genes and genetic disorders developed by the US National Center for Biotechnology Information
The US National Kidney and Urologic Diseases has a page about hemolytic uremic syndrome
The Wikipedia has a page about HUS (note that Wikipedia is a free online encyclopedia that anyone can edit)
PMCID: PMC1626556  PMID: 17076561
10.  Severe haemolytic anaemia due to cold anti-'i' antibodies associated with cytomegalovirus infection. 
Postgraduate Medical Journal  1990;66(775):392-394.
A 66 year old patient with multiple myeloma and monoclonal cryoglobulinaemia who developed a severe haemolytic anaemia following a cytomegalovirus infection is reported. The presence of a high titre of anti-'i' cold antibody of IgM subclass is demonstrated. Anti-'i' antibody disappeared when complement-fixation antibody titres against cytomegalovirus decreased. Various pathogenetic mechanisms involved in the development of haemolytic anaemia associated with cytomegalovirus infection are discussed. To our knowledge, this is the first case described in the English language publications associating severe haemolytic anaemia with an anti-'i' antibody after a cytomegalovirus infection in an immunocompromised patient.
PMCID: PMC2426850  PMID: 2164663
11.  Autoimmune haemolytic anaemia in a child with MHC class II deficiency. 
Archives of Disease in Childhood  1994;71(4):339-342.
A 3 year old Turkish girl is described who was suffering from major histocompatibility complex (MHC) class II deficiency syndrome, which is characterised by the lack of expression of HLA class II antigens on mononuclear cells. The presence of HLA class II genes was demonstrable at the DNA level. Combined immunodeficiency was indicated by hypogammaglobulinaemia and the absence of delayed type hypersensitivity on skin testing. Further, she was unable to produce specific antibodies towards foreign antigens and suffered from recurrent pulmonary, gastrointestinal, and septic infections from the third month of life. The clinical course was complicated by a Coombs test positive haemolytic anaemia due to the production of autoantibodies against the rhesus "e' antigen, a non-glycosylated protein antigen. Haemolysis could be controlled by oral steroid treatment. This case is of interest as it shows that despite the absence of HLA class II antigens and combined immunodeficiency autoimmune reactions with production of specific autoantibodies directed to protein antigens are possible.
PMCID: PMC1030015  PMID: 7979529
12.  Infectious mononucleosis with secondary cold agglutinin disease causing autoimmune haemolytic anaemia 
BMJ Case Reports  2009;2009:bcr12.2008.1390.
This case report describes a 20-year-old woman whose initial clinical, laboratory, and radiological presentation suggested obstructive jaundice. However, she was subsequently found to be suffering from autoimmune haemolytic anaemia resulting from an Epstein–Barr virus infection complicated by cold agglutinin disease. The patient went on to make a complete clinical recovery after discharge.
PMCID: PMC3028242  PMID: 21894246
13.  Haemolytic uremic syndrome following fire ant bites 
BMC Nephrology  2014;15:5.
Haemolytic-uremic syndrome (HUS) is a severe, life-threatening disease with symptoms such as haemolytic anaemia, renal failure, and a low platelet count. Possible aetiology includes bacterial infections, medication, post-hematopoietic cell transplantation, pregnancy, autoimmune disease, and acquired immunodeficiency syndrome.
Case presentation
We report the case of a 21-year-old healthy man who developed acute renal failure caused by HUS. Typical symptoms of HUS combined with severe uraemia developed following a large local reaction after suspected Solenopsis invicta (fire ant) bites. He was successfully treated with plasma exchange and achieved complete recovery of renal function.
This is the first case illustrating a serious systemic reaction of HUS to fire ant bites, and highlights this severe complication in patients who sustain fire ant bites.
PMCID: PMC3890644  PMID: 24400942
Fire ant; Haemolytic-uremic syndrome; Plasma exchange; Renal failure; Venomous insects
14.  Phenacetin-induced haemolytic anaemia 
Journal of Clinical Pathology  1971;24(6):537-541.
Haemolytic anaemia, rarely severe, is a common yet often unrecognized complication of the prolonged use or abuse of phenacetin-containing analgesics. Irregularly contracted (pyknocytes) or fragmented erythrocytes (schistocytes) are commonly present in the peripheral blood in this form of anaemia. It is emphasized that their recognition during screening of blood films may reveal patients previously unsuspected of analgesic abuse and at a stage before the development of the more serious complication of nephropathy. Fourteen such patients, detected during the last 18 months, are briefly described and the pathogenesis and laboratory features of the anaemia reviewed.
PMCID: PMC477089  PMID: 5094685
15.  Is a tricuspid annuloplasty ring significantly better than a De Vega's annuloplasty stitch when repairing severe tricuspid regurgitation? 
A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether tricuspid valve (TV) repair with an annuloplasty ring leads to an improved outcome over a conventional suture annuloplasty for patients with severe tricuspid incompetence. Altogether, 306 papers were found using the reported search, of which 14 presented the best evidence to answer the clinical question. The author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses of these papers are tabulated. We found seven studies supporting the use of ring annuloplasty over De Vega's suture annuloplasty. Five studies found no significant difference in outcome between the two techniques. We found only two studies supporting the use of De Vega's suture annuloplasty over ring annuloplasty. Therefore, most of the tabulated studies show good evidence in support of ring annuloplasty compared with De Vega's suture repair for treatment of moderate to severe TV regurgitation. One cohort study identified a 20.4% postoperative failure rate for tricuspid regurgitation (TR) repair and concluded non-application of ring as a predictor for reoperation. One cohort study with a mean follow-up of 17 months reported success rates as high as 97% with ring annuloplasty for TV regurgitation. One large cohort study of 2277 patients with TR who had undergone repair reported a sustained reduction in TR and the rate of recurrence in a 5-year echocardiographic follow-up. One cohort study of 129 patients concluded that ring annuloplasty has the lowest rate of recurrence compared with De Vega's suture repair. An old randomized controlled trial (RCT) on the subject also reported a similar result to the mentioned studies. In contrast, we reviewed one recent study and four older studies and found no significant difference between the two techniques. We reviewed one study that reported De Vega's suture repair as a superior technique to ring annuloplasty. We conclude that there is good evidence supporting ring annuloplasty over conventional De Vega's suture annuloplasty.
PMCID: PMC3380971  PMID: 22457189
Tricuspid valve; Suture; Cardiac valve annuloplasty
16.  Pegylated interferon de novo-induce autoimmune haemolytic anaemia in chronic hepatitis C patient 
BMJ Case Reports  2011;2011:bcr0620114400.
A 55-year-old Egyptian woman with chronic hepatitis C undergoing treatment with pegylated interferon (Peg-IFN) alfa-2a plus ribavirin was referred to our hospital on November 2010 with prolonged easy fatigability and an attack of syncope; she had no prior history of autoimmune disorders or allergy. Laboratory investigations documented the presence of Peg-IFN induced autoimmune haemolytic anaemia and autoimmune thyroiditis. Intravenous γ globulin (IVGG) failed to correct the autoimmune process; on the other hand steroid therapy dramatically corrected both haematological and thyroid values, and step down the immune process. Our report indicated that Peg-IFN de novo-induce autoimmune haemolysis, documenting a previous report. IVGG failed to step down the immune process in our case.
PMCID: PMC3158342  PMID: 22688484
17.  Cold autoimmune haemolytic anaemia secondary to Epstein Barr virus infection presenting with peripheral gangrene; case report 
Thrombosis Journal  2012;10:4.
A sixty year old male presented with dark urine, symptomatic anaemia and peripheral gangrene following cold exposure. Investigations revealed that he had haemolysis and serological evidence of recent Epstein Barr virus infection. Although acrocyanosis is commonly associated with cold agglutinin disease, gangrene is a rare complication. Management of secondary cold agglutinin disease is mainly supportive.
PMCID: PMC3403954  PMID: 22513360
Cold autoimmune haemolytic anaemia; Haemolysis; Gangrene
18.  Prevalence of HIV-related autoimmune haemolytic anaemia in Lagos, Nigeria 
Despite a high frequency of anaemia, a positive direct antiglobulin test (DAT) and bone marrow hyperplasia HIV-infected patients, lack of reticulocytosis may cause underdiagnosis autoimmune haemolytic anaemia (AIHA) in them. This study was carried out to determine the prevalence of autoimmune haemolytic anaemia in HIV-infected patients and to compare the haematological/immunological characteristics of subjects with anaemia and those without.
Materials and Methods:
A total of 350 HIV-infected subjects attending the Lagos University Teaching Hospital who consented were recruited for the study. This included 250 subjects with anaemia (haemoglobin concentration <10 g/dl) as cases and 100 subjects without anaemia as controls. Five milliliters of venous blood drawn from each subject was used for the full blood count, reticulocyte count and DAT.
Subjects with anaemia had lower mean CD4 cell count (284.3 cells/μl) and higher mean reticulocyte per cent (1.5%) than the non-anaemic subjects. The frequency of reticulocytosis was higher in female subjects than in males. Only 0.8% (2 of 250) of the study group screened positive to DAT, p = 0.0339. None of the subjects in control group screened positive to DAT.
Autoimmune haemolytic anaemia is a rare complication of HIV infection in our geographical location.
PMCID: PMC4071666  PMID: 24970973
Autoimmune haemolytic anaemia; direct antiglobulin test; haemoglobin; reticulocytosis
19.  Haemolytic anaemia after cisplatin treatment. 
Normochromic or normocytic anaemia is a common side effect of treatment with cisplatin. Two patients treated with cisplatin 100 mg/m2 in combination with vinblastine, bleomycin, and actinomycin D developed haemolytic anaemia. Neither patient had evidence of haemolysis before treatment, and in both cases severe haemolytic anaemia developed after several courses of cisplatin and when the cancer had regressed almost completely. The importance of haemolysis in the development of anaemia after cisplatin treatment has not been investigated fully and further studies are needed.
PMCID: PMC1505958  PMID: 6788166
20.  Haemolytic anaemia associated with splenic haemangiomata. 
Postgraduate Medical Journal  1988;64(748):152-154.
We describe a 33 year old woman who developed unexplained haemolytic anaemia following renal transplantation and who was found to have multiple splenic haemangiomata. This case demonstrates that splenic haemangiomata may be a cause of haemolytic anaemia in the absence of abnormalities in coagulation.
PMCID: PMC2428792  PMID: 3050938
21.  The Co-Existence of Pure Red Cell Aplasia and Autoimmune Haemolytic Anaemia in a Child with Malignant Lymphoma 
The association between pure red cell aplasia (PRCA) and autoimmune haemolytic anaemia (AIHA) has rarely been reported. PRCA represents an isolated process, characterized by normochromic, normocytic anaemia, reticulocytopenia and erythroid hypoplasia in the bone marrow, and may be attributable to infection with Parvo virus B19. AIHA is a condition in which peripheral red blood cell destruction is induced by the presence of autoantibodies. However, the co-existence of these conditions is very rare, since only few cases of PRCA and AIHA associated with malignant lymphoma (ML) were reported. A case of PRCA and AIHA was detected and described, for the first time in Malaysia, in a 10-year-old child suffering from non-Hodgkin lymphoma from the Department of Haematology, Universiti Sains Malaysia. Following the induction course of chemotherapy, the patient turned anaemic, with tendency for red cell clumping, reticulocytopenia and anisocytosis. AIHA was suspected in spite of the weak Coomb reaction obtained. The bone marrow aspirate revealed the presence of giant pronormoblasts, suggesting PRCA. Serological tests for Parvo virus and other viruses were negative.
PMCID: PMC3349402  PMID: 22605959
pure red cell aplasia; autoimmune haemolytic anaemia; malignant lymphoma
22.  Treatment of hemopericardium caused by mitral balloon valvuloplasty with activated factor VII: a case report 
The use of mitral balloon valvuloplasty as a percutaneous intervention for mitral stenosis has been shown to be efficacious. Cardiac tamponade is a rare but serious complication of this procedure. Despite the low incidence of this event, cardiac tamponade is well-reported in the literature. The management strategy of this complication involves pericardial drainage and correction of coagulopathy, followed by surgical exploration if these interventions fail. With this case report, we demonstrate the successful application of activated factor VII in the management of bleeding after balloon valvuloplasty that persisted despite the standard treatments described above.
Case presentation
Our patient was a 31-year-old Yemenite man with no significant past medical history, who presented with progressively worsening dyspnea on exertion and limited functional capacity over the last few years. A transesophageal echocardiogram revealed severe mitral stenosis, which was treated with a mitral valve valvuloplasty. The procedure was complicated by significant mediastinal bleeding that did not respond to routine maneuvers, which included pericardiocentesis and correction of coagulopathy. Our patient was evaluated for surgical intervention but responded to treatment with activated factor VII.
Factor VII may be used in the treatment of refractory mediastinal bleeding secondary to mitral valvuloplasty prior to attempting surgical repair, and therefore may spare the patient the morbidity associated with surgery.
PMCID: PMC3930136  PMID: 24467868
Complications and management; Factor VIIa; Hemopericardium; Mediastinal bleeding; Mitral balloon valvuloplasty
23.  Necrotizing arteritis with giant cells associated with haemolytic anaemia 
Journal of Clinical Pathology  1965;18(5):588-592.
Necrotizing arteritis with giant cells, involving the aorta, pulmonary and coronary arteries, and coronary, splenic, and renal arterioles was found at necropsy in a 74-year-old male who had died with severe haemolytic anaemia associated with cold haemagglutinins.
The clinical and pathological features of this case are shared by well-recognized forms of necrotizing angiitis, in particular giant cell arteritis, but do not conform satisfactorily with any of these disorders. Cold agglutinin haemolytic anaemia in this instance suggests the possibility of an autoimmune aetiology.
PMCID: PMC473006  PMID: 4378652
24.  Nationwide study of haemolytic uraemic syndrome: clinical, microbiological, and epidemiological features 
Archives of Disease in Childhood  2001;85(2):125-131.
AIMS—To establish the incidence and aetiology of haemolytic uraemic syndrome (HUS) in Australia and compare clinical and microbial characteristics of sporadic and outbreak cases.
METHODS—National active surveillance through the Australian Paediatric Surveillance Unit with monthly case notification from paediatricians, July 1994 to June 1998. Children under 15 years presenting with microangiopathic haemolytic anaemia, thrombocytopenia, and acute renal impairment were identified.
RESULTS—Ninety eight cases were identified (incidence 0.64 per 105 children <15 years/annum and 1.35 per 105 children <5 years/annum). Eighty four were associated with diarrhoea (64 sporadic, 20 constituting an outbreak) and 14 were atypical. Shiga toxin producing Escherichia coli (STEC) O111:H− was the most common isolate in sporadic HUS and caused the outbreak. However O111:H− isolates from outbreak and sporadic cases differed in phage type and subtyping by DNA electrophoresis. STEC isolates from sporadic cases included O26:H−, O113:H21, O130:H11, OR:H9, O157:H−, ONT:H7, and ONT:H−. STEC O157:H7 was not isolated from any case. Only O111:H− isolates produced both Shiga toxins 1 and 2 and possessed genes encoding E coli attaching and effacing gene (intimin) and enterohemolysin. Outbreak cases had worse gastrointestinal and renal disease at presentation and more extrarenal complications.
CONCLUSIONS—Linking national surveillance with a specialised laboratory service allowed estimation of HUS incidence and provided information on its aetiology. In contrast to North America, Japan, and the British Isles, STEC O157:H7 is rare in Australia; however, non-O157:H7 STEC cause severe disease including outbreaks. Disease severity in outbreak cases may relate to yet unidentified virulence factors of the O111:H− strain isolated.

PMCID: PMC1718875  PMID: 11466187
25.  A rare presentation of micro-angiopathic haemolytic anaemia in a critically ill patient: a case report 
Cases Journal  2009;2:6294.
A 36-year-old woman presents to hospital peri-arrest with hypertension, sustained loss of consciousness following a tonic clonic seizure and a micropathic haemolytic anaemia on blood film. After initial resuscitation, more specialised treatment was instigated as the diagnosis became clearer but all was not as it first seemed. This case demonstrates the importance of re-examination, especially in the critically ill, in conjunction with unusual laboratory tests in order to eventually reach a rare diagnosis of a rare presentation.
PMCID: PMC2740215  PMID: 19829782

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