Unveiling the masked native blood group following thalidomide therapy in a thalassemia patient: A 5 WHY analysis approach.

Blood grouping discrepancy in patients with hematological disorders can occur due to red cell sensitization following transfusion, transplantation, and pregnancy or pre-analytical errors. Prompt initiation of root cause analysis is vital to avoid complications of wrong blood transfusion. We present an unusual case of Rh mismatched grouping report of 24 year old female thalassemia patient being managed in our hospital since 2015. Her current type and screen were observed as O Rh D negative with negative antibody screen while the historical blood group was O Rh D positive. The pre-analytical errors were ruled out and blood grouping performed from fresh sample also demonstrated as O Rh D negative despite antigen enhancement techniques and had no recent transfusion history. We sought to reason out the possibilities for discordant Rh grouping report, historical and present group through "Funnel based problem solving 5 WHY analysis" approach. The review of the past clinical history revealed that the patient had undergone Rh mismatch bone marrow transplant (Rh D positive donor and Rh D negative recipient) at 5 years of age which soon resulted in graft failure. Yet, she continued to receive Rh D positive blood thereafter with no development of anti-D which explains the historical blood group. Recently the patient was started on thalidomide, the Hb F inducer drug, which helped in maintaining her hemoglobin level between 9 and 10 g/dl without transfusion support for two months. This allowed unmasking of native Rh D negative blood and the review of clinical history played a significant role in resolution of grouping discrepancy.

Efficacy of cascade plasmapheresis in comparison with conventional therapeutic plasma exchange for relapsed atypical hemolytic uremic syndrome: A case report.

Atypical hemolytic uremic syndrome (aHUS) is a rare and life-threatening disease that is associated with high mortality and morbidity. The incidence of aHUS is about 1 or 2 cases per 1,000,000 per year. Etiology can be either familial or sporadic. The pathogenesis of aHUS involves dysregulation of the alternative complement pathway, with predisposing mutations in complement genes. aHUS has a poor prognosis and a gradual or a relapsing (30%-86%) clinical course. The disease may present at any age but is mostly seen in children and young adults. Therapeutic plasma exchange (TPE) is one of the primary modalities of treatment in aHUS. This report presents the utilization of cascade plasmapheresis and its advantages over TPE in a patient with relapsed aHUS. There was a 73% decrement in antifactor H antibody levels following cascade plasmapheresis.

Passenger lymphocyte syndrome in a bidirectional ABO-mismatched renal transplant.

Passenger lymphocyte syndrome (PLS), a subtype of graft-versus-host disease, is due to the production of antibodies by the donor "passenger" B lymphocytes against recipient's red cells. It is a rare disorder encountered mostly in ABO blood group-mismatched solid organ transplantation. The present case report illustrates the clinical presentation and the mode of management of PLS in a bidirectional ABO-incompatible renal transplantation. A 43-year-old male diagnosed with chronic kidney disease Stage 5-D (diabetic nephropathy) Type-2 hypertension with ischemic heart disease underwent ABO bidirectional-mismatched renal transplantation. The blood group of the patient was B Rh D positive and that of the donor (patient's wife) was A Rh D positive. In the pretransplantation phase, immunoglobulin G anti-A titer was 64 by column agglutination method, which was subsequently brought down to 4 by therapeutic plasma exchange and immunosuppression. Good graft function was established in the posttransplantation phase, but a significant drop in the hemoglobin (Hb) was noted. A fall in Hb, peripheral smear findings suggestive of hemolysis, and direct antiglobulin test positivity along with raised lactate dehydrogenase suggested the diagnosis of PLS; the patient was managed successfully for the same by transfusion of O blood group packed red blood cell transfusion and immunosuppression. PLS is a rare but important cause of immune-mediated hemolytic anemia in ABO-mismatched transplants.