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Primary Hepatic lymphoma (PHL) is a very rare malignancy representing approximately 0.016% of all cases of Non Hodgkin Lymphoma (NHL). It oftens presents in elderly persons with signs and symptoms of hepatitis, primary liver cancer or metastatic tumor. In this case report, we present a 58 year old lady with poor performance status who presented with obstructive jaundice, epigastric pain and bilious vomiting. Abdominal imaging revealed a large hepatic mass that was pathologically confirmed primary hepatic diffuse large B cell lymphoma without bone marrow involvement. Because of severe jaundice, she was managed with Rituximab, cyclophosphamide and prednisolone based therapy, but succumbed to neutropenic fever following chemotherapy.

Mantle cell lymphoma (MCL) is a distinct type of non-Hodgkin’s lymphoma that commonly affects extra nodal sites. The most commonly affected sites are bone marrow, gastrointestinal tract and Waldeyer’s ring, however, skin is rarely involved. We report a case of 56 year-old patient with MCL, exhibiting multiple large maculopapular skin rashes and skin ulcers. Histopathological examination demonstrated dilated and congested vessels in the dermis without any atypical cells. Our patient showed significant improvement of skin lesions with a combination chemotherapy and debridement. Awareness of skin manifestations of MCL is essential to establish the diagnosis and for timely administration of appropriate treatment.

Introduction Janus kinase2 (JAK2) V617F mutation in myeloid cells has been reported from the majority of patients with Polycythemia vera (PV). Materials & Methods A cross sectional study was conducted to review clinical records of 93 cases of erythrocytosis who were evaluated for JAK2V617F mutation by Amplification Refractory Mutation System polymerase chain reaction at our institution. The significance of this mutation in the context of clinical & hematological features in cases that fulfilled World Health Organisation 2008 diagnostic criteria for PV was investigated. Results 66(71%) patients fulfilled the WHO diagnostic criteria for PV. Erythrocytosis could be attributed to secondary causes in 20(21.5%) cases and in seven (7.5%) cases, erythrocytosis was idiopathic. The JAK2V617F mutant allele was detected in 61 of the 66 patients (92.4%) with 19(31.1%) showing homozygosity. A comparison between JAK2V617F mutation positive and JAK2V617F wild type PV patients at presentation revealed that the JAK2V617F mutation positive patients were older(P=0.05), with significantly lesser mean values of hemoglobin (P= 0.02). Compared with heterozygote counterparts, JAK2V617F homozygotes showed significantly higher hemoglobin (P=0.03), incidence of thrombosis(P=0.04) and transformation to myelofibrosis(P=0.02). Conclusions This is the largest Indian study correlating JAK2V617F mutation status with clinicohematologic characteristics in PV patients and reveals that prevalence of JAK2V617F mutational status is 92.4% in PV patients with 31.1% homozygosity. JAK2V617F homozygosity is associated with significantly higher hemoglobin values, and, confers higher risk of thrombosis and progression to myelofibrosis.

Introduction: Inherited bleeding disorders are characterized by the absence or reduced level of clotting factors, and the clinical manifestations vary according to the type and magnitude of the deficient factor. Aim: To study the clinical presentation of these rare inherited coagulation factor disorders in a tertiary care hospital and to compare the data from those reported in other populations. Methods: Sixty-seven patients, who presented to the Department of Hematology, All India Institute of Medical Sciences, New Delhi, were evaluated retrospectively from 2005 to 2011. The tests performed included platelet count, prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time (TT), factors assay and clot solubility test in 5 M urea. Factors VIII, IX and XI assays were aPTT based while factors II, V, VII and X assays were PT based. Results: Male to female ratio was 2:1. The median age of onset of the first episode of bleeding was at 6 months (range, from birth to 20 years) whereas the median age of presentation to our hospital was 9 years (range, 2 months to 54 years). The most common deficient factor was factor X (43.28%), followed by factor XIII deficiency (26.86%) and factor VII (10.4%). Conclusion: There is a wide gap between the initial manifestation of the bleeding disorder and first presentation to the tertiary care hospital for assessment and treatment. Factor X deficiency is the most common among these rare coagulation disorders in our population, whereas factor VII deficiency is more common in Iranian and North American population.

Madurella mycetomatis is the most common fungal agent causing eumycetoma. The commonest clinical presentation of the infection is the appearance of multiple sinuses with discharge of grains, which are the colonies of the fungus. It is an emerging fungal infection among transplant recipients and has not been reported following stem cell transplantation. We report here a case of aplastic anemia who developed madura foot, caused by Madurella mycetomatis, following allogenic peripheral blood stem cell transplantation. The diagnosis was made by the examination of discharged black grains under microscope which reavaled presence of septate hyphae and the culture of the discharged granules grew Madurella mycetomatis. The patient was treated with voriconazole followed by excision of the lesion, which resulted in complete recovery. Considering the increasing number of patients undergoing stem cell transplantation for various hematological diseases, the implications of this fungal infection should be recognized as delay in treatment may be life-threatening.