Beenu Thakral

Phenotype frequencies of blood group systems (Rh, Kell, Kidd, Duffy, MNS, P, Lewis, and Lutheran) in north Indian blood donors

BACKGROUND We here report the first study of antigen and phenotype frequencies of various blood group systems by gel technology in north Indian blood donors. STUDY DESIGN AND METHODS A total of 1240 regular repeat voluntary north Indian blood donors of O blood group were included for red cell antigen typing of Rh (D, C, E, c, e) and Kell (K) blood group systems. Out of these, 317 donors were randomly selected for typing of other blood group antigens: Jk(a), Jk(b), k, Kp(a), Kp(b), Fy(a), Fy(b), M, N, S, s, Le(a), Le(b), P(1), Lu(a), Lu(b) and Xg(a). Calculations of antigen and phenotypes frequencies were expressed as percentages and for allele frequencies under the standard assumption of Hardy-Weinberg equilibrium. RESULTS Out of 1240 O group blood donors, 93.39% were Rh D and 5.56% were K positive. Amongst Rh antigens, e was the most common (98.3%) followed by D, C (84.76%), c (52.82%) and E (17.9%) with DCe/DCe (R(1)R(1), 43.8%) being the most common phenotype. In Kell blood group system, we found k antigen to be 100% and a rare phenotype Kp (a+b+) was found in 0.95% of the donors. For Kidd and Duffy blood group systems, Jk (a+b+) and Fy (a+b-) were the most common phenotypes (49.21% and 43.85%, respectively). In the MNS blood group system, M+N+S+s+ (19.55%) was the most common whereas M-N+S+s- (1.26%) was least common phenotype found. We found rare Lu (a+b+) and Lu (a-b-) phenotypes in 0.95% and 3.15% of the donors, respectively. Xg(a) antigen was seen in 86.67% and 62.6% of female and male donors, respectively. CONCLUSIONS Knowledge of red cell antigen phenotype frequencies in a population is helpful in terms of their ethnic distribution, in creating a donor data bank for preparation of indigenous cell panels, and providing antigen negative compatible blood to patients with multiple alloantibodies.

First report from India of haemolytic disease of newborn by anti-c and anti-E in Rh (D) positive mothers

Abstract In India, at the majority of the transfusion centers, antenatal antibody screening is done only in Rh (D) negative mothers. We report here two cases of haemolytic disease of newborn (HDN) by maternal alloimmunization to anti-c in one case, and both anti-c and anti-E in the second case. Both women were Rh (D) positive mothers and HDN was detected postnatally by a positive direct antiglobulin test (DAT) in their newborns. These cases highlight the importance of routine antenatal antibody screening even in Rh (D) positive females. A close follow-up throughout pregnancy is required if irregular antibodies are present so that antigen negative, crossmatch compatible blood can be provided in a timely manner for intra-uterine or exchange transfusions.

Scope and application of therapeutic apheresis: Experience from a tertiary care hospital in North India

BACKGROUND We present here our experience with therapeutic apheresis (TA) performed for various indications, clinical response and complications in a tertiary care center over last 10 years. STUDY DESIGN AND METHODS Present study is a retrospective analysis of 492 TA procedures performed for 125 patients from January 2000 to December 2009. For each patient: age, gender, weight, clinical indication, pre-procedure hematological profile and ionized calcium levels were recorded. For every procedure following parameters were analyzed: type of venous access (central/peripheral), volume of blood and plasma processed, amount of anticoagulant used, procedure duration, blood flow rate, type of replacement fluid given, response to therapy and adverse reactions. RESULTS Of 492 TA procedures, 68.8% were performed for neurology, 20.8% hematology-oncology, 9.6% renal and 0.8% for rheumatology patients. Therapeutic plasma exchanges (n=464; 94.3%) and therapeutic cytapheresis (n=28; 6.7%) were performed in 113 and 12 patients, respectively. Majority of patients belonged to ASFA category I and II (n=124; 99.2%). The overall response rate was 84%, with encouraging response in TTP (100%), aHUS (81.8%) and in neurological disorders (88.4%). Adverse events were reported in 52.8% of patients in 14.83% of procedures. CONCLUSION Our results of TPE in neurological disorders and in atypical hemolytic uremic syndrome are encouraging and it is a cost effective alternative to IvIg in neurological disorders. Currently, there is a need for establishment of an Indian apheresis registry to understand the scenario of TA across the country and in the expansion of appropriate and applicable indications for TA in our setting.

Hemolytic disease of newborn due to anti-Jkb in a woman with high risk pregnancy

This case illustrates the importance of blood group antibodies in antenatal serology other than Rh system as a cause of hemolytic disease of newborn (HDN). In India, antenatal antibody screening is done at majority of transfusion centers in only Rh (D) negative mothers. In this multigravida woman with high risk obstetrical history, an antenatal antibody screening by indirect antiglobulin test (IAT) was not performed as she was Rh (D) positive. Postnatal work up for the pathological jaundice in the neonate revealed that red cell alloimmunization had occurred due to anti-Jk(b). We conclude that antenatal antibody screening should be done in all pregnant women irrespective of the D antigen status to detect and manage red cell alloimmunization to any other clinically significant blood group antigens.

Prognostic Impact of CD5 Expression in Diffuse Large B‐cell Lymphoma in Patients Treated with Rituximab‐EPOCH

CD5‐positive (CD5+) diffuse large B‐cell lymphoma (DLBCL) represents 5%‐10% of all DLBCL cases, which has been associated with a poorer prognosis in patients treated with R‐CHOP. Prognostic impact of CD5 expression in patients with DLBCL treated with R‐EPOCH has not been evaluated.

Therapeutic Plateletpheresis in a Case of Symptomatic Thrombocytosis in Chronic Myeloid Leukemia

Abstract:  Extreme thrombocytosis is a frequent feature in myeloproliferative disorders which can predispose a person to thrombotic complications. As opposed to other myeloproliferative disorders, symptomatic thrombocytosis is rare in chronic myeloid leukemia. We describe a second case report of chronic myeloid leukemia (Ph chromosome positive) in a patient in chronic phase on hydroxyurea who presented with sudden onset digital cyanosis of the left hand, giddiness, headache and malaise due to extreme thrombocytosis. A 67% global reduction in the platelet count from 1553 × 109/L to 513 × 109/L after two therapeutic plateletpheresis procedures was seen. There was simultaneous improvement in all symptoms except cyanosis on the tip of the middle finger that progressed to dry gangrene. Dramatic reduction in the platelet count and ablation of symptoms by therapeutic plateletpheresis is an effective therapy and should begin as soon as possible.

Encrusted Cystitis Secondary to Corynebacterium glucuronolyticum in a 57-Year-Old Man Without Predisposing Factors.

Encrusted cystitis is a rare condition characterized by encrustation of the bladder mucosa with associated chronic inflammation induced by urea-splitting bacterial infection--most commonly, Corynebacterium urealyticum. Moreover, it usually occurs in immunocompromised patients, especially recipients of renal transplants or patients with a history of previous urological procedures. Due to the rarity of the entity and the slow growth of Corynebacterium species, appropriate treatment is often delayed due to difficulties in diagnosis and resistance to numerous antibiotics. We report a case of encrusted cystitis caused by Corynebacterium glucuronolyticum, another urea-splitting microbe, in a 57-year-old previously healthy Caucasian man with no known predisposing factors. The timely diagnosis and management in this otherwise healthy patient was facilitated by characteristic imaging, cystoscopy, and histologic findings confirmed by results of prolonged urine cultures and 16S ribosomal RNA (rRNA) gene sequencing of the microbe.

Castleman's disease in the orbit of a 17-year-old girl: A case report

Castlemans disease is an atypical lympho proliferative disorder comprising hyaline vascular elements, plasma cells, or a mixture of both, which can present in unicentric or multicentric fashion. Resection of unicentric lesions is typically curative, but multicentric disease, also characterized by constitutional symptoms and a poorer prognosis, often requires treatment with chemotherapy, radiation, steroids, or immune modulators. Castlemans disease is rarely diagnosed in the orbit. The authors present the clinical and histopathological findings of a 17-year-old who was found to have a focal lesion in her orbit. She was successfully treated with surgical resection and was free of disease recurrence or other sequelae at 10-months follow up.

“Soccer ball” cells to “donut” cells: An unusual case of Richter syndrome

![Figure][1] A 56-year-old man presented with relapsed chronic lymphocytic leukemia (CLL) with 90% bone marrow (BM) involvement, “soccer ball” morphology (panel A, hematoxylin and eosin stain; panel B, Wright-Giemsa stain), and the following immunophenotype: CD5+/CD19+/CD20dim+/CD38+/ZAP-

Well-differentiated systemic mastocytosis showed excellent clinical response to imatinib in the absence of known molecular genetic abnormalities: A case report

Introduction: Well-differentiated systemic mastocytosis (WDSM) is a rare, recently recognized provisional subvariant of systemic mastocytosis (SM). We report a case of WDSM that showed excellent clinical and cutaneous response to imatinib in the absence of known molecular genetic abnormalities. Clinical Findings/Diagnoses: We present a 24-year-old woman with childhood onset of skin manifestations that progressed to mediator-related systemic events, and a gastrointestinal tract mastocytoma. A subsequent bone marrow examination showed WDSM. Treatment with imatinib resulted in complete resolution of cutaneous lesions and systemic symptoms, which relapsed with the discontinuation of the drug. Targeted next-generation sequencing-based mutation analysis did not demonstrate any mutations in the coding regions of KIT or other genes commonly associated with myeloid neoplasms. Conclusions: The diagnosis of WDSM is challenging in the absence of spindle-shaped mast cells, CD2 or CD25 expression, and KIT D816 mutation. This case illustrated the need for recognizing this unique variant of SM for diagnostic and therapeutic implications.