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.
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.
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.
Haemolytic anaemia; Surgical repaired aortic dissection and aortic regurgitation
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.
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.
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.
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.
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.
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.
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.
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.
Haemolytic anaemia of obscure aetiology is a common complication of pregnancy in Nigeria. Treatment with antimalarials and folic acid is usually followed by a rapid remission, but response is slow in about 25% of patients and haemolysis continues uncontrolled in about 5%. The administration of prednisolone to six patients with uncontrolled haemolysis was followed by rapid recovery in five and possible benefit in one. Risks of prednisolone therapy to the mother appear to be slight and outweighed by the risks of continued severe anaemia and frequent blood transfusions. There seemed to be no appreciable increase of fetal loss compared with that in anaemic pregnancies not treated with prednisolone.
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.
Cold autoimmune haemolytic anaemia; Haemolysis; Gangrene
A middle-aged man was found to have autoimmune haemolytic anaemia. Seven years after the first manifestations of the anaemia, he developed jaundice without haemolysis and a diagnosis of primary sclerosing cholangitis was made by endoscopic retrograde cholangiography. Crohn's colitis was later confirmed by X-rays and colonoscopy. This association is unique to the best of our knowledge and suggests that genetic and immunological mechanisms may be involved in the pathogenesis of these diseases.
A patient, who underwent aortic valve replacement, developed a transient, autoimmune haemolytic anaemia in association with a significant rise in titre of antibodies against cytomegalovirus. A serological survey of blood donors indicated that the source of infection was fresh blood transfused during and shortly after surgery. A number of cases of short-lived haemolytic anaemia after large volume blood transfusion with cardiac surgery may be explained by this mechanism. It may be preferable to use only blood that has been screened for evidence of recent cytomegalovirus infection if post-perfusion complications of this disease are to be avoided.
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.
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.
We describe a case of severe autoimmune haemolytic anaemia developing in newly diagnosed ulcerative colitis of moderate severity. Full immunosuppression with steroids and azathioprine failed to bring about a full remission and a splenectomy was performed which resulted in a remission enabling immunosuppression to be discontinued. This case is compared with three previously described cases of autoimmune haemolytic anaemia associated with severe colitis requiring splenectomy.
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.
pure red cell aplasia; autoimmune haemolytic anaemia; malignant lymphoma
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.
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.
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.
The association of chronic active hepatitis with haemolytic anaemia is well known. Both conditions may respond to steroid therapy which, in common with other causes of suppressed T-lymphocyte function, predispose to many types of infection. A case is described in which transient Listeria monocytogenes bacteraemia occurred and the patient recovered without antimicrobial therapy.
A 71 year old woman presented with hypocalcaemia in association with autoimmune haemolytic anaemia and pernicious anaemia. The hypocalcaemia resolved only when the haemolytic anaemia and pernicious anaemia were successfully treated. The possible pathogenic mechanisms are discussed.
The case histories of two patients with penicillin-induced haemolytic anaemia are presented. One had received 20 mega units a day for 18 days, the other had received 20 mega units a day for two days and then 12 mega units a day for 25 days, before the haemolytic anaemia was diagnosed. Both had previously had courses of penicillin. A strongly positive direct antiglobulin reaction which appeared to be mainly due to IgG antibody was one of the main diagnostic features, and free IgG antipenicillin antibody was found in the serum of both patients. The haemolysis appeared to Lessen as soon as the drug was stopped, and the direct antiglobulin test became negative in 66–77 days.
Twelve additional reported cases are reviewed. All had received high doses of penicillin and all had had penicillin previously. The lowest dose recorded was 10 mega units a day for 26 days. The incidence of anti-penicillin antibodies in a hospital population is given, and the mechanism of this type of haemolytic anaemia is discussed. Penicillin-induced haemolytic anaemia should be suspected in any patient receiving penicillin in high doses in whom there is a fall in the haemoglobin level.
Glucose phosphate isomerase (GPI) deficiency with severe haemolysis and hydrops fetalis was found in the first child of unrelated, healthy Caucasian parents. The child died at 3 hours. Both parents were found to have 50% of normal red cell GPI activity and qualitative tests on their red cells and white cells showed that each was heterozygous for a different GPI variant allele associated with enzyme deficiency. Tests on the placenta showed that the propositus was a 'compound' heterozygote. Examination of amniotic cells obtained by amniocentesis on the mother at 28 weeks in her second pregnancy led to the prenatal diagnosis of GPI deficiency. This second child, a 'compound' heterozygote at the GPI locus indistinguishable from the first, was successfully treated by immediate exchange transfusion and subsequent blood transfusions.