A Rare Case of Primary Plasma Cell Leukaemia with Monosomy X.

Primary Plasma Cell Leukaemia (pPCL) is a very rare and aggressive subtype of plasma cell dyscrasias with a very poor outcome and characterized by presence of >2x109/l circulating plasma cells. Overall incidence being 1%-2% of all malignant plasma cell diseases and 0.9% of all acute leukaemias. Patients with primary and secondary PCL have similar clinical features but differences do exist. Here we report a rare and interesting case diagnosed as primary PCL in a 44-year-old female with unusual clinical presentation and complex karyotyping. Her serum electrophoresis showed a monoclonal IgG component and immunohistochemistry of bone marrow plasma cells showed CD 38 positive and CD 20 negative. The patient initially responded partially for combination chemotherapy, but succumbed after 20 days of diagnosis. We are presenting this case to highlight the importance of early diagnosis of such haematological malignancies in settings where treatment options like stem cell transplantation are limited.

Effectiveness of Pooled Platelet Transfusion in Concordant and Discordant Groups among Dengue Patients.

INTRODUCTION: Dengue affects more than 50 million people per year and is one of the most common causes of severe thrombocytopaenia. Thrombocytopaenia is a common complication of dengue and other viral fevers apart from malaria, typhoid, leptospirosis, leukaemia and megaloblastic anaemia. A platelet count of <20,000/µl is characteristically seen in dengue haemorrhagic fever and dengue fever. It results from immune complex mediated platelet destruction or bone marrow suppression. Severe thrombocytopaenia <10,000/µl is one of the indications for prophylactic platelet transfusion therapy to prevent haemorrhage. AIM: To evaluate the effectiveness of transfusion of ABO compatible and ABO incompatible pooled platelet units in severe thrombocytopaenia cases. MATERIALS AND METHODS: In this study ABO compatible and incompatible pooled platelet units were transfused to serologically confirmed dengue cases having thrombocytopaenia with or without bleeding manifestations. Each of the adult patients received 4-6 units of pooled platelet concentrates prepared from random donor whole blood suspended in plasma for severe thrombocytopaenia. Pre and post transfusion platelet counts were compared. Children aged less than 12 years, pregnant women and patients with splenomegaly those on ayurvedic and homeopathic therapy, recipients of packed red cells on the same day of platelet transfusion and recipients of multiple platelet transfusions within 24 hours were excluded from the study. RESULTS: The median post transfusion platelet increments (PPI) and corrected count increments (CCI) at 4hour post transfusion were 25,000/µL (5,000-80,000/µL) and 18,000/µL (range 8,000/µL- 47,500/µL) respectively among the responders. Median PPI and CCI at 24 hours were 45,000/µL and 28,863/µL among the responders. The median CCI at 4 hour post transfusion among the non-responders was 850/µL and at 24hours was 1,425/µL. At 24 hours responders showed significantly higher PPI as compared to non responders. The average platelets transfused were 4units in case of responders and 8 units in case of non-responders. CONCLUSION: ABO identical and compatible pooled platelet transfusions were more successful in increasing the post transfusion platelet counts as compared to ABO incompatible pooled platelets.

Eccrine Porocarcinoma of the Scalp: A Rare Case Report with Review of Literature.

Eccrine porocarcinoma (EPC) is a potentially lethal neoplasm of the skin that arises from the intraepidermal portion of the eccrine sweat glands. It was previously known as eccrine adenocarcinoma or malignant eccrine poroma. It commonly occurs between 60 to 80 years of age, usually arising from hands and feet. However, few case reports on involvement of rare sites such as scalp, face and eyelids are present in literature. We herein report an unusual case of porocarcinoma arising on the right parieto occipital region of scalp in a 29-year-old young female patient. To the best of our knowledge, there have been fewer than 20 cases of porocarcinoma arising on scalp with fewer than 10 cases seen in younger age group reported previously in the literature.