Beta-thalassemia is one of the most prevalent autosomal disorders, which affect more than 400,000 newborn per year worldwide. In India, the carrier rate of beta-thalassemia varies from 3-17%. The overall rate of alloimmunization in thalassemia patients has been reported to be 5-30% in the world, which is mostly contributed by the alloimmunization to minor blood group antigen. Among Asians, the incidence of red cell alloimmunization is 22%. The recommended treatment for beta-thalassemia major is regular blood transfusion every 3 to 4 weeks. The development of anti-red cell antibodies (alloantibodies and/or autoantibodies) can significantly complicate transfusion therapy. Alloantibodies are commonly associated with red cell hemolysis. Red cell autoantibodies appear less frequently, but they can result in clinical hemolysis called autoimmune hemolytic anemia (AIHA), and in difficulty in cross-matching blood. Patients with autoantibodies may have a higher transfusion rate and often require immunosuppressive drugs or alternative treatments including intravenous immunoglobulin (IVIg) and rituximab (anti-CD20 monoclonal antibody).
Thalassemia major; auto immune hemolytic anemia; alloimmunization
Transfusion-related acute lung injury (TRALI) is a serious clinical syndrome associated with the transfusion of plasmacontaining blood components. Recently, TRALI has come to be recognized as the leading cause of transfusion-related mortality. This complication typically presents as shortness of breath, hypoxemia, hypotension, fever, and non cardiogenic pulmonary edema, occurring within 6 h after transfusion. Although the mechanism of TRALI has not been exactly known, it has been associated with human leukocyte antigen antibodies and with biologically active mediators in stored cellular blood components. We, hereby, present a case of a patient with dengue fever who developed acute lung injury (ALI), presumably TRALI, after transfusion of platelet concentrates. He was treated with supportive measures and mechanical ventilation. Greater knowledge and increased awareness especially amongst the clinicians regarding TRALI is needed for prevention and treatment of this potentially severe complication of blood/component transfusion.
Acute lung injury; blood transfusion; non cardiogenic pulmonary edema; transfusion-related acute lung injury
Donor notification and post-donation counseling is an essential role of blood bank. If a donor is reactive for any marker, the blood bank counselor, informs the donor and advices him/her to report to the blood bank for further counseling and management. The counselor at our blood bank informed a young female voluntary donor to be reactive for HIV both with ELISA as well as NAT. When the donor reported to blood bank, the repeat testing was negative and no history of high risk behavior could be elicited. The hospital information system (HIS) records were checked again immediately for clarification and showed consistency with her demographic profile. But when her manual records and donor questionnaire were retrieved, showed information displayed in the HIS system was wrongly interpreted by the counselor. In this era of information technology being highly advanced, the role of manual record keeping is still the gold standard.
Donor notification; hospital information system; reactive donor
A 66-year-old female patient presented with complaints of abdominal discomfort, pigmentation and numbness of both lower limbs for 3 years duration. On examination, she had erythema of the face and palms. Investigations revealed high hemoglobin (Hb), hematocrit (Hct) and erythropoietin. Ultrasonography abdomen showed large uterine fibroid. As there are increased tendencies of thromboembolic episodes in patients undergoing surgeries with such high Hb and Hct, a target to achieve a Hb of 15g/dl and Hct of 45 was set in the patient. Repeated phlebotomies were done over 10 days with oral hydration only and the Hb was brought down to 18 g/dl on the day prior to surgery. On the day of surgery, pre-operative phlebotomy was done so as to remove 2 units of 350 ml blood and was transfused intraoperatively to combat blood loss. Post-operatively Hb was 12.4 g %. Patient was discharged on the 10th post-operative day with further follow-up evaluations being uneventful.
Leiomyomatous erythrocytosis; perioperative hemodilution; phlebotomy
Plasma is used to correct coagulopathies, but not all coagulation abnormalities are clinically significant enough to require correction before an invasive procedure. We report an 82-year-old female who, in response to a mildly prolonged INR of unknown etiology, was unnecessarily transfused with plasma in advance of elective surgery. The patient suffered a moderately severe transfusion reaction, including hives and voice hoarseness, which caused a 4-week delay in her surgery. This delay and adverse reaction could have been avoided had the principles of evidence based plasma therapy, which we herein review, been followed and if the etiology of the mildly elevated INR been investigated before the day of her surgery.
Allergic reaction; guidelines; plasma; transfusion
Immune hemolysis is one of the adverse effects that can occur following solid organ transplantation. Understanding the clinical settings and the various causes is necessary for prompt diagnosis and appropriate management. One such condition is passenger lymphocyte syndrome (PLS). This case report describes the case of a 27-year-old male renal allograft recipient of the B-positive blood group who received a kidney from an O-positive donor. Postoperatively, the patient showed declining hemoglobin (Hb) level and was transfused with B-group packed RBCs (PRBCs), following which there was steep fall in Hb level. A request for PRBCs was sent to the blood bank and this time cross-match with B-group PRBCs showed incompatibility. The patient's RBCs were found to be strongly DAT (direct anti-globulin test) positive and the eluate showed the presence of anti-B with a titer of 32. Thus, diagnosis of probable PLS was made. The patient was managed with methylprednisolone, plasmapheresis and O-group PRBCs. Gradually his condition improved and was discharged in stable condition.
Antibody titer; DAT; hemolysis; passenger lymphocyte syndrome; transplantation
Guillain-Barre syndromé (GBS) is an autoimmune disorder. It is rare in pregnancy as there is a decrease in cell-mediated immunity. A case of 28-year-old pregnant woman who presented with acute flaccid quadriplegia suffering from GBS is discussed in this study. She was treated with plasma exchange in her immediate post-partum period. The management of GBS in pregnancy has been discussed.
Guillain-Barré Syndrome in pregnancy; plasma exchange; therapeutic plasma exchange
Severe hemolysis was observed in a critically ill patient with G6Pd deficiency where the causative trigger could not be identified. We describe one young patient with severe hemolysis treated with two cycles of plasmapheresis which proved to be an effective tool in the treatment. The patient presented with diffuse pain abdomen, vomiting, yellowish discoloration of sclera and skin and acute breathlessness. Hemoglobin 5.4 mg/dl and total (T) serum bilirubin 17.08 mg/dl: Direct (D) 4.10 mg/dl and Indirect (I) 12.98 mg/dl. Subsequently patient started passing black color urine. As the patient developed severe hemolysis and the trigger agent of hemolysis was unknown, two cycles of plasmapheresis were performed with the aim to remove unknown causative agent. Consequently no trace of hemolysis was found and patient stabilized. Plasmapheresis can be used to treat G6PD deficient patients with severe hemolysis due to unidentified trigger agent.
Acute hemolytic anemia; Glucose-6-phosphate dehydrogenase deficiency; plasmapheresis; therapeutic plasma exchange
It appears that delayed hemolytic transfusion reactions may occur several days after the administration of donor red cells is true even though they have been shown to be compatible in cross match tests by the antiglobulin technique. A specific case was observed in our center, which confirms the fact. The patient was a 37-year-old male suffering from intermediate β-thalassemia. He had a history of two previous transfusions, with unknown transfusion reaction. In the last transfusion, laboratory data showed: Hb 7.8 g/dL and Hematocrit (Hct) 24.2%. The patient received two units of cross matched, compatible concentrated red blood cells (RBCs). After eight days a severe reaction was observed with clinical evidence of tachycardia, fatigue, fever, back pain, chest discomfort, jaundice, nausea and anorexia. Accordingly delayed hemolytic transfusion reaction was suspected, and anti-RBC antibodies were tested. Laboratory tests revealed the presence of three alloantibodies: Anti-N, anti-S, anti-K, and a monospecific autoanti-JKb.
Alloantibody; autoanti-JKb; delayed hemolytic transfusion reaction; β-Thalassemia
Autoimmune Hemolytic Anemia (AIHA), a very infrequent condition which represents a group of disorders in which presence of autoantibodies directed against self-antigens leads to shortened red cell survival. Till date, a very few cases of AIHA in Malaria patients are reported worldwide but still AIHA should be considered a relatively rare cause of anemia in malaria. A 20 year male presented with intermittent fever since seven days and yellowish discoloration of urine and sclera since 5 days. He was transfused three units of blood at a private clinic before one month. On examination, pallor, icterus and spelnomegaly were present. Hemoglobin (Hb) was 3.2 gm% and peripheral smear revealed ring forms of both Plasmodium vivax and Plasmodium falciparum. Serum LDH and Serum billirubin (Indirect and Direct) were high. This patient’s blood group was B +ve with positive autocontrol. Indirect Antiglobulin Test (IAT), antibody screening and antibody identification were pan-positive with reaction strength of +4 against each cell. Direct Antiglobulin Test was +4 positive anti IgG and negative with anti C3. He was treated with Artesunate and methylprednisone. Least incompatible, saline washed O Neg and B neg red cells were transfused on the 2nd day of starting treatment. Hb was raised to 6.1 gm% on 4th day. Patient was discharged on 9th day with Hb 7.0 gm% with oral tapering dose of steroids. In the above case, patient was suffering from high grade malarial parasitemia with co-existing autoimmune RBC destruction by IgG auto-antibodies which led to sudden drop in Hb and rise in serum LDH and indirect billirubin. Least incompatible packed red cells along with antimalarials and steroids led to clinical improvement. So far, one case report each from India, Korea, Canada and Germany and one case series report of three cases from India have been reported. Under-reporting or rarity of this phenomenon may be accountable for this.
Autoimmune Hemolytic Anemia; autoantibodies; malaria
We are reporting a rare case of severe hemolytic disease of newborn (HDN) with Bombay phenotype mother. A retrospective study of a case with severe haemolytic disease of newborn with Bombay phenotype mother was done. Blood grouping, antibody screening, and lectin study was done on the blood sample of the baby and mother to confirm the diagnosis. Hematological and biochemical parameters were obtained from the hospital laboratory information system for the analysis. Blood group of the baby was A positive, direct antiglobulin test was negative. Blood group of the mother was confirmed to be Bombay phenotype, Hematological parameters showed all the signs of ongoing hemolysis and the bilirubin level was in the zone of exchange transfusion. Due to the unavailability of this rare phenotype blood unit, baby was managed conservatively. Anticipating the fetal anemia and HDN with mothers having Bombay phenotype and prior notification to the transfusion services will be of great help in optimizing the neonatal care and outcome.
Blood grouping; Bombay phenotype; hemolytic disease of newborn
Alloimmunization against the Rhesus-D (RhD) antigen still remains as a major cause of hemolytic disease of fetus and newborn (HDFN). Determination of paternal RhDzygosity is performed by molecular testing and is valuable for the management of alloimmunized pregnant women. A 30-year-old pregnant woman with AB negative blood group presented with two consecutive abortions and no history of blood transfusion. By application of the antibody screening, identification panel, and selected cells, she was found to be highly alloimmunized. RhDzygosity was performed on her partner and was shown to be homozygous for RhD. The sequence- specific priming-polymerase chain reaction used in this report is essential to establish whether the mother requires an appropriate immunoprophylaxis or the fetus is at risk of HDFN.
Rhesus-Dzygosity; hemolytic disease of fetus and newborn; anti-D
Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant inherited disorder of the vascular system. It can be asymptomatic but when symptomatic most common presentation being epistaxis. It can involve any organs of the body like lungs, skin, liver brain, GI mucosa etc. We are reporting a case of HHT presented to us with dyspnea and severe anemia. He had arteriovenous malformations of different visceral organs and telangiectasia of skin along with presence of similar history in first-degree relatives.
Anemia; arteriovenous malformation; dyspnea; hereditary hemorrhagic telangiectasia
Thrombotic thrombocytopenic purpura (TTP) occurs more commonly in women and so can be associated with pregnancy. The time during pregnancy with greatest risk for development of TTP is near term and during the post partum period. TTP occurring in early trimester is uncommon and is also associated with great maternal and fetal mortality. We report a successful outcome of pregnancy in a woman with TTP in early first trimester who was treated with therapeutic plasma exchange.
First trimester; plasmapheresis; pregnancy; thrombotic thrombocytopenic purpura
Maternal allo-antibody production is stimulated when fetal red blood cells are positive for an antigen absent on the mother's red cells. The maternal IgG antibodies produced will pass through the placenta and attack fetal red cells carrying the corresponding antigen. Allo-immune hemolytic disease of the fetus and newborn caused by anti-E rarely occurs.
We report two cases of anti-E hemolytic diseases in neonates. One of the neonates had severe hemolysis presenting with severe anemia, thrombocytopenia, and conjugated hyperbilirubinemia, while the other had moderate anemia and unconjugated hyperbilrubinemia. Although both the neonates were treated by phototherapy and intravenous immunoglobulin, one of them received double volume exchange transfusion.
There appeared to be an increase in the occurrence of hemolytic disease of the fetus and newborn caused by Rh antibodies other than anti-D. In this case report, both patients presented with anemia and hyperbilirubinemia but were successfully treated, with a favorable outcome.
Allo-antibody; anti-E; hemolytic disease of the fetus and newborn
Transfusion management of patients’ alloimmunized against high-prevalence erythrocyte antigens is often problematic in emergency situations. Gerbich (Ge) is very common blood group system and Gerbich–2 (Ge–2) antigen present in high frequency and outside Papua New Guinea population, Ge–2 negative population almost nil. To manage such kind of problems with real emergencies, implementation of rare donor registry program, cryopreservation of red cells of rare donors and biological cross matching to assess significance of these antibodies is warranted.
Alloantibodies; alloimmunization; Gerbich antigen; high frequency antigens
Allergic reactions occur commonly in transfusion practice. However, severe anaphylactic reactions are rare; anti-IgA (IgA: Immunoglobulin A) in IgA-deficient patients is one of the well-illustrated and reported causes for such reactions. However, IgE-mediated hypersensitivity reaction through blood component transfusion may be caused in parasitic hyperimmunization for IgG and IgE antibodies.
We have evaluated here a severe anaphylactic transfusion reaction retrospectively in an 18year-old male, a known case of cerebral malaria, developed after platelet transfusions. The examination and investigations revealed classical signs and symptoms of anaphylaxis along with a significant rise in the serum IgE antibody level and IgG by hemagglutination method. Initial mild allergic reaction was followed by severe anaphylactic reaction after the second transfusion of platelets.
Based on these results, screening of patients and donors with mild allergic reactions to IgE antibodies may help in understanding the pathogenesis as well as in planning for preventive desensitization and measures for safe transfusion.
Anaphylactic transfusion reaction; IgE mediated allergic transfusion reaction; investigation of transfusion reaction; platelet transfusion reactions
Bombay phenotype is extremely rare in Caucasian with an incidence of 1 in 250,000. When individuals with the Bombay phenotype need blood transfusion, they can receive only autologous blood or blood from another Bombay blood group. Transfusing blood group O red cells to them can cause a fatal hemolytic transfusion reaction. In this study, we report a case with the rare Bombay blood group that was misdiagnosed as the O blood group and developed a hemolytic transfusion reaction. This highlights the importance of both forward and reverse typing in ABO blood grouping and standard cross-matching and performing standard pretransfusion laboratory tests in hospital blood banks.
ABO blood-group system; blood group incompatibility; blood transfusion; Bombay phenotype; transfusion reaction
A case of hyperhemolysis in a 2-year-old boy with β thalassemia major was noted. After several transfusions, he developed hyperhemolysis with a positive (C3d only) direct antiglobulin test (DAT) and no clinically significant RBC allo- or auto-antibodies. (There was a weak cold antibody, showing a narrow thermal range). Because there was no significant improvement with steroid and immunoglobulin infusions, cyclophosphamide therapy was tried with notable success.
β-thalassemia; direct antiglobulin test; hyperhemolysis; transfusion
Anti-M is a fairly common naturally occurring antibody with rarely causing hemolytic transfusion reactions or hemolytic disease of the newborn. Most anti-M are not active at 37°C and can generally be ignored in transfusion practice. However, we did not find this antibody to be fairly common and detected only two cases of anti-M in the past three years. We describe these two cases; one ‘immunizing’ type and other ‘naturally occurring’ and review the literature. The immunizing type was reactive at 37°C as well as AHG phase of testing with IgG component, and showing dosage effect while the other was ‘naturally occurring’ reactive well below 37°C. Though rare, sometimes these antibodies can be of clinical significance when the antibody detected is reactive at 37°C and AHG phase. When the antibody is active at 37°C, M antigen negative cross match compatible red cell unit should be given.
Anti-M; immunizing; naturally occurring
A case is described here of drug-induced immune hemolytic anemia (DIIHA) due to cefotetan administered to a post-partum woman who received the drug for infection prophylaxis at the time of caesarean section. Renewed fatal hemolysis occurred when the drug was given a second time 12 days after the first dose. The initial immunohematologic findings included a positive direct antiglobulin test (DAT) due to IgG and complement coating of the patient’s RBCs as well as an eluate that did not react with RBCs in the absence of drug. The antibody was drug-dependent, reacting with both drug-coated RBCs as well as when the drug was added to a mixture of her serum and donor RBCs. Cefotetan has been a common cause of this uncommon problem. The clinical features of cefotetan DIIHA, classification of drug-induced antibodies, and the differential diagnosis of a positive DAT are briefly discussed.
Cefotetan; direct antiglobulin test; drug-induced immune hemolytic anemia
Cell separators in India are routinely used for plateletpheresis, peripheral blood stem cell collections and therapeutic plasma exchange. Therapeutic leukapheresis, particularly as an emergency procedure, has been uncommonly performed and reported. Here, a case of a 53-year-old male, diagnosed with acute myeloid leukemia subtype M5 (AML M5) with hyperleukocytosis, who underwent emergency leukaphereis, is reported. After two procedures, there was a decrease of WBC count by 85%, which enabled cytotoxic therapy to be initiated.
Emergency; hyperleukocytosis; leukapheresis