Sudha S Bhat

Nasal NK/T cell lymphoma presenting as a lethal midline granuloma.

Nasal NK/T cell lymphomas are aggressive, locally destructive, midfacial, necrotizing lesions. The nonspecific clinical symptoms constitute a major stumbling block in the early diagnosis and management of these lymphomas. We report here a case of probable nasal NK/T cell lymphoma in an apparently healthy male that progressed rapidly in a short span of time and was managed subsequently with chemotherapy and external beam irradiation with which the lesion regressed.

Multiple neural fibrolipomas with macrodactyly

Neural fibrolipoma is an uncommon tumor-like lesion that involves the upper extremity and usually arises in the median nerve. It is associated with macrodactyly in one-third of the cases. A 3-year-old girl presented with increasing size of fingers of both the hands since birth. Clinical examination revealed macrodactyly of two fingers of the right hand and three fingers of the left. Surgical reduction was performed and microscopy of the biopsy specimen established the diagnosis of neural fibrolipoma. Knowledge of the clinicopathological features is necessary for accurate diagnosis and treatment of this rare entity.

Endometrial stromal nodule with smooth muscle differentiation

Uterine tumors composed of a prominent component of smooth muscle and endometrial stroma (so-called stromomyoma) are distinctly uncommon. This article describes the morphological features of one such tumor discovered as an incidental finding in a hysterectomy specimen of a 49-year-old lady with a clinical diagnosis of dysfunctional uterine bleeding. Morphological and immunohistochemical (IHC) evaluation were performed and a final diagnosis of endometrial stromal nodule with smooth muscle differentiation was rendered.

A rare case of haemolytic disease of newborn with Bombay phenotype mother

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.

Linear accelerator: A reproducible, efficacious and cost effective alternative for blood irradiation

BACKGROUND The dedicated devices for blood irradiation are available only at a few centers in developing countries thus the irradiation remains a service with limited availability due to prohibitive cost. OBJECTIVE To implement a blood irradiation program at our center using linear accelerator. MATERIALS AND METHODS The study is performed detailing the specific operational and quality assurance measures employed in providing a blood component-irradiation service at tertiary care hospital. X-rays generated from linear accelerator were used to irradiate the blood components. To facilitate and standardize the blood component irradiation, a blood irradiator box was designed and fabricated in acrylic. Using Elekta Precise Linear Accelerator, a dose of 25 Gy was delivered at the centre of the irradiation box. Standardization was done using five units of blood obtained from healthy voluntary blood donors. Each unit was divided to two parts. One aliquot was subjected to irradiation. Biochemical and hematological parameters were analyzed on various days of storage. Cost incurred was analyzed. RESULTS Progressive increase in plasma hemoglobin, potassium and lactate dehydrogenase was noted in the irradiated units but all the parameters were within the acceptable range indicating the suitability of the product for transfusion. The irradiation process was completed in less than 30 min. Validation of the radiation dose done using TLD showed less than ± 3% variation. CONCLUSION This study shows that that the blood component irradiation is within the scope of most of the hospitals in developing countries even in the absence of dedicated blood irradiators at affordable cost.

A rare case of missing antibody due to anti‐snake venom

Immunohematologic tests are influenced by various factors, including patient age, disease condition, and treatment. Solving a case of blood grouping discrepancy is often challenging. Here we report the first case of a missing antibody due to the administration of snake venom antiserum for the management of a child after snake bite. A request for routine blood grouping was sent from the pediatric intensive care unit for a 6-year-old child. She was admitted with a snake bite, suspected to be from a cobra. She had cellulitis and compartment syndrome of right leg that required fasciotomy. On admission, her hemoglobin level was 13.2 g/dL, prothrombin time was 20.5 seconds (control, 14.8 sec), and activated partial thromboplastin time was 37.1 seconds (control, 34 sec). She was discharged from the hospital after 2 weeks with complete recovery. The result of initial blood grouping is shown in Fig. 1. Further testing with anti-A,B done using test tubes was negative. Repeat blood typing gave similar results with a fresh sample. Blood grouping was carried out in an automated blood grouping machine using ABD and reverse diluent cards (AUTO/ VUE, Ortho-Clinical Diagnostics, Inc., Raritan, NJ). To enhance the assay, reverse grouping was done in test tubes and kept at 4°C for 1 hour along with the autocontrol and group O cells. Under these conditions, a weak positive reaction was seen with “A” cells and no agglutination was seen with the autocontrol and group O cells. All laboratory tests were carried out in accordance with the manufacturer’s insert. Parents’ ABO grouping was determined using AUTO/VUE and both the parents were group O. The direct antiglobulin test, the red blood cell antibody screening using gel cards, and the three-cell antibody screening panel (DiaMed AG, Cressier sur Morat, Switzerland) showed negative results. On reviewing the patient’s history, it was found that she was treated with more than 50 vials of anti-snake venom—polyvalent (ASVS-ASIA; Bharat Serums and Vaccines Ltd, Thane, India). To check the effect of anti-snake venom on blood group antibodies, we selected a group O serum sample from a normal blood donor and performed reverse typing in the presence and absence of anti-snake venom. As shown in Fig. 2, there was no reaction with A cells in the presence of anti-snake venom, whereas normally a 4+ reaction is expected. From these observations, it was inferred that the blood group discrepancy is attributable to anti-snake venom and the group was reported as group O D+. According to the manufacturer’s details, each milliliter of anti-snake venom neutralizes not less than the following quantities of standard venom tested in mice by the intravenous route: cobra (Naja naja), 0.6 mg; common krait (Bungarus caeruleus), 0.45 mg; Russell’s viper (Vipera russelli), 0.6 mg; and saw scaled viper (Echis carinatus), 0.45 mg. Because anti-snake venom is a concentrated preparation of serum globulins obtained by fractionating blood from healthy hyperimmunized horses, false positivity during immunohematologic testing can be expected but a false-negative result is unusual. Although anti-snake venom binds to and neutralizes venom, some constituents of equine-derived anti-snake venom may also act as antigens to neutralize anti-A. In an earlier study, A or A-like substances were present in every specimen of horse serum investigated and B substance was not demonstrable. So A substance is a property of horse serum itself, and it is likely that these substances in anti-snake venom neutralized the anti-A. At the time of

Case report: CT features of cherubism

Cherubism is a rare hereditary disease of non-neoplastic origin seen in childhood and characterized by bilateral bony enlargement of the jaws.[1] It causes diffuse, bilateral, and multilocular expansion of the mandible and/or maxilla and has a characteristic radiographic and histopathological appearance.[1,2] In this case report, we describe the CT features of this uncommon condition in a 14-year-old girl.

Further evidence for naturally occurring anti Jk(a) antibodies.

Sir, Antibodies to red cell antigens are considered naturally occurring when there is no obvious source stimulus such as blood transfusion, injection or pregnancy. However, these antibodies are produced as an immune response to some unknown environmental antigens such as pollen grains and other parts of bacterial membranes.[1] The commonly mentioned naturally occurring antibodies belong to the ABO, Hh, Ii, Lewis, MN, P blood group systems. Anti-Kidd antibodies are notorious for confounding features and as a causal for delayed hemolytic transfusion reactions. Even strong AntiKidd antibodies may become undetectable after few weeks or months of immune stimulus and are frequently known to show dosage effect. Anti-Kidd antibodies are often diffi cult to detect and usually are commonly found in combination with other antibodies refl ecting the low immunogenicity of the Kidd antigen.[2] There are numerous reports of these antibodies. Anti Jkb is rarer than anti Jka and there are only two reported cases of naturally occurring antiKidd antibodies in the literature.[3,4] The present case confi rms the possibility of naturally occurring anti-Kidd antibodies.

Mixed field agglutination: Unusual causes and serological approach

Mixed field agglutination in serology usually describes the presence of two populations of red cells. Here we present images with two uncommon reasons for mixed field reaction. In both the cases, we observed mixed field reaction during pretransfusion testing using column agglutination technology [Figure 1]. Relevant patients history was obtained and further immunohematological work-up was carried out in both the cases to look for the possible cause. In the first case, there was no history of blood transfusion, direct antiglobulin test was negative. Antibody screening cells showed pattern and the antibody identified was anti-M. However, all the panel cells with heterozygous expression of M antigen showed mixed field agglutination. Presence of anti-M antibody was confirmed by enzyme treatment and phenotyping the patient for M antigen. In second case, uniform mixed field agglutination was observed with the antibody screening cells including the autocontrol. Direct antiglobulin test was negative. However, we suspected the interference of test results by fibrin residues. To confirm and to resolve the problem, we incubated patients clotted sample at 37°C for 30 min and used well-separated serum sample for repeat antibody screening. As shown in the image of the second case, mixed field reaction resolved completely and on cross-matching the problem did not recur. Figure 1 Mixed field agglutination: Unusual causes and serological approach Knowing all the possible causes of mixed field reaction would help to resolve and interpret the results accordingly. Anti-M is generally a cold reacting antibody and its reaction strength may vary because of dosage. Antibodies to Lutheran and Sda antigens may show mixed field appearance but it is unusual for anti M antibodies.[1] However in the present case, mixed field reaction was noticed only with the panel cells having heterozygous expression of M antigen. Serum separation though a very simple technique has a great importance on the outcome of the immunohematological results. As illustrated in this case, failure to adhere to proper serum separation technique would unnecessarily create confusion and delay the transfusion support to the patient.

Desmoplastic Small Round Cell Tumour in a Young Woman with Widespread Metastasis and Peritoneal Caking

Desmoplastic Small Round Cell Tumour (DSRCT) is a rare, highly aggressive, mesenchymal tumour that arises from the peritoneal cavity. It is commonly seen in adolescent and young adult males and its occurrence in females is uncommon. We are reporting here a rare case of DSRCT in a young woman, which clinically masqueraded as an ovarian malignancy, with metastasis to liver, lung, spleen and peritoneum. The cytologic findings, Histomorphological and immunohistochemical features have been discussed, with a brief review of literature.