Karan Saluja

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.

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.

Radiologic and histologic presentation of male mammary myofibroblastoma

Mammary myofibroblastoma is a rare mesenchymal neoplasm that typically presents in older men and women. Less commonly, these benign tumors may also occur in soft tissues located outside of the breast, in which case they are referred to as mammary-type myofibroblastomas. The histologic composition of this benign spindle cell tumor can be markedly varied. We present a case of a large mammary myofibroblastoma in a male patient and discuss the typical imaging and histologic makeup of these tumors.

Platelet audit: Assessment and utilization of this precious resource from a tertiary care hospital.

Background: To assess the appropriate utilization of platelet transfusions [random donor platelets (RDP) and single donor platelets (SDP)]; a six-month retrospective audit was carried out in a tertiary care hospital. Materials and Methods: A six-month retrospective platelet audit was carried out from May to October 2005 to estimate its preparation, appropriate utilization and wastage rate. Patients demographics, transfusion triggers and episodes and ABO and Rh (D) group specific or non-group specific transfusions were also assessed. Results: About 5525 units of platelets [PRP-PC, 3,813 (69%); BC-PC, 983 (17.8%); PRP, 648 (11.7%) and SDP 81 (1.5%)] were prepared and transfused to 853 patients (RDP to 814 patients and SDP to 39 patients) in 2,093 transfusion episodes. Adult and pediatric hemato-oncology were the main user specialties utilizing 39.1 and 87.6% of the RDPs and SDPs prepared. Of the patients receiving RDPs, 95% were transfused ABO and Rh (D) group specific platelets whereas 100% SDPs transfusions were of group specific platelets. 88% of prophylactic platelet transfusions were appropriate as per the recommended BCSH guidelines. However, 12% of the prophylactic platelets were transfused inappropriately in cardiopulmonary bypass (CPB) surgeries with normal platelet counts and no evidence of bleeding related to platelets. Out of 5,444 RDPs prepared 1,585 (29.11%) units were not utilized. Conclusions: Regular audit of blood and blood components is a must so that necessary remedial measures can be taken to maximize appropriate and judicious utilization of each component.

Misdiagnosed MYH9 related inherited macrothrombocytopenia with an inadvertent splenectomy

consistent with T-ALL/LBL. These two cases raise the possibility of a rare overlap syndrome. To our knowledge this is the first report of phenotyping TCR Vb expression of the precursor T-cell infiltrate of thymoma. Further work is needed to determine if TCR Vb-restricted thymocyte populations are a common and reproducible finding in thymoma, or only present in a minority of cases. Correlation with disease stage would also be of interest. Further work investigating the prevalence of Vb restricted T-cell precursor populations infiltrating thymoma would be of use, particularly if they offer prognostic value. Immunopathologists, anatomical pathologists and treating physicians should be aware of a possible T-ALL/LBL and thymoma overlap syndrome.

Crystal storing histiocytosis associated with marginal zone B-cell lymphoma: A rare initial clinical presentation diagnosed by fine-needle aspiration

Sir, Crystal storing histiocytosis (CSH) is a rare manifestation of B-cell lymphoproliferative disorders characterized by intracytoplasmic accumulation of crystallized immunoglobulins within the macrophages.[1] CSH can be generalized involving multiple sites or can manifest as a localized mass lesion, most commonly in the head and neck region. It equally involves both males and females; with a wide age distribution (17-81 years).[2] We report an interesting case of localized CSH associated with marginal zone B-cell lymphoma involving a retroperitoneal lymph node at the left renal hilum. The patient was a 77-year-old male who presented with a slow growing mass at the renal hilum over a period of 3 years, with an increase in size from 4.1 cm to 7.0 cm. The complete blood counts, urine analysis, and comprehensive metabolic profile on a 6-monthly regular follow-up during the 3 year period had always been normal. The patient initially declined undergoing an invasive procedure. However, due to the progressive increase in mass size, he consented, and a fine-needle aspiration (FNA) and core biopsy of the mass was performed. The FNA smears demonstrated a monomorphic population of mononuclear cells, lymphoglandular bodies, and scattered histiocytes. The histiocytes demonstrated low nuclear to cytoplasmic ratio, small round regular eccentric nucleus, and abundant refractile, dense amorphous, needle to rhomboid shaped crystals within the cytoplasm. No mast cells or plasma cells were noted in the background [Figure ​[Figure1a1a and ​andb].b]. This cytologic appearance of the histiocytes was suggestive of CSH, which prompted further workup for a lymphoproliferative process. The histological sections of the core biopsy demonstrated monomorphic lymphoid aggregates admixed with sheets of oval to polygonal histiocytes filled with eosinophilic refractile crystals within the cytoplasm pushing the nucleus to the side [Figure 1c]. Immunohistochemical stain performed on the tissue core biopsy for kappa and lambda light chains demonstrated monoclonal staining pattern for kappa light chains in the lymphocytes as well as in the cytoplasmic crystals within the histiocytes [Figure 1d]. The histiocytes were highlighted with positive staining for CD68 [Figure 1e]. Cyclin D1 immunostain was negative. Flow cytometry performed on the aspirated material demonstrated a clonal B-cell lymphoid population with the following immunophenotype: CD45+, CD19+, CD20+, CD3−, CD5−, CD10−, and kappa light chain restricted. No paraprotein was detected in the serum or urine electrophoresis. No other lymphadenopathy or splenomegaly was observed on imaging. A diagnosis of localized CSH associated with a nodal marginal zone B-cell lymphoma was rendered. The patient declined further therapy and was lost to follow-up. Figure 1 (a and b) Fine-needle aspiration of the mass demonstrate monomorphic lymphocyte population admixed with scattered histiocytes in a clear background. Inset: Histiocyte with refractile cytoplasmic crystals (Diff Quik, ×100 and ×200). (c) ... On review of the literature, approximately 80 cases have been reported to date with the majority of them (90%) associated with lymphoproliferative or plasma cell disorder including multiple myeloma (32%), lymphoplasmacytic lymphoma (24%), monoclonal gammopathy of undetermined significance (21%), B-cell lymphoma (15%) or plasma cell dyscrasia/neoplasm, not otherwise specified (6%).[2] CSH is usually associated with kappa light chain restriction as seen in the present case due to high intrinsic stability or a loss of proteolytic site on these light chains. The associated heavy chain is variable. Ultrastructurally, the crystals are dense, membrane bound and elongated, rectangular and/or rhomboid in configuration.[2] The exact mechanism for crystal formation is not well-known and may involve factors ranging from overproduction to impaired secretion or excretion of the immunoglobulin chains. The common sites involved in generalized CSH include bone marrow (97%), liver (47%), lymph nodes (44%), spleen (44%), and kidney (38%), whereas, head and neck region (parotid and thyroid gland, cornea, or otolaryngeal mucosa) is the most commonly involved in localized CSH.[2,3,4,5] Generalized CSH is usually associated with poor prognosis, regardless of the type of underlying lymphoproliferative process, however, in localized CSH the prognosis is variable depending upon the extent of tissue involved and the underlying condition of the patient. In general, the prognosis and overall survival in patients with localized CSH associated with marginal zone lymphoma is comparable to age and sex matched normal patient control, as described in a recent review by Zhang and Myers.[4] We herewith, describe a rare presentation of a lymphoproliferative process due to the presence of background CSH initially diagnosed on a FNA. A similar report of mucosa-associated lymphoid tissue (MALT) lymphoma involving the parotid gland has been described in an 81-year-old female on FNA, demonstrating immunoglobulin crystals within histiocytes admixed with monocytoid B-cells and plasma cells.[3] MALT lymphoma in these mucosal locations is analogous to marginal zone B-cell lymphoma involving the lymph node described in the present case. Crystals in CSH are usually described most often of immunoglobulin origin, which are associated with an underlying lymphoproliferative or plasma cell disorder. However, non-immunoglobulin associated CSH also exist, with the differential diagnosis include adult rhabdomyoma, granular cell tumor, Langerhans cell histiocytosis (LCH), Gauchers disease, malakoplakia, drug-induced CSH (clofazimine), and hereditary cystinosis.[6,7,8,9,10] The crystals in adult rhabdomyoma represent tropomyosin and are thus positive for various muscle makers such as desmin, muscle specific actin, and/or myoglobin; granular cell tumor shows positive staining for S-100; nuclei of histiocytes in LCH are folded or have grooves resembling coffee bean, stain positive for CD1a and S-100, and are usually associated with eosinophils in the background. In Gauchers disease, the cytoplasm of the histiocyte has striated appearance (crumpled tissue paper) due to the deposition of beta-glucocerebroside and stain positive for PAS. Malakoplakia is most often found in the urinary tract, less commonly in the head and neck region, composed of sheets of CD68-positive epithelioid histiocytes (von Hansemann histiocytes) and scattered pathognomonic Michaelis-Gutmann bodies which contain Gram-negative bacteria with laminated dystrophic calcium and iron deposits. Clofazimine is a drug used to treat leprosy and can present as crystals within the histiocytes, usually limited within the bowel wall and mesenteric lymph nodes, and show prominent red birefringence under polarized light on frozen sections. Thus, in summary, CSH is a well-recognized entity with an accumulation of intra-cytoplasmic crystalline immunoglobulin light chain deposits within non-neoplastic histiocytes and its presence should alert for a workup of a lymphoproliferative process. In addition, an extensive search including imaging, bone marrow biopsy, and serum/urine electrophoresis should be followed to evaluate the extent of involvement of the lymphoproliferative process associated with CSH. The present case adds CSH associated with rare marginal zone B-cell lymphoma to that list.