Congenital fibrinogen deficiency is an inherited disorder due to genetic mutations with diverse presentations arising from reduced fibrinogen levels (hypofibrinogenemia), absence of fibrinogen in circulation (afibrinogenemia), abnormal functioning (dysfibrinogenemia) or both reduced levels and abnormal functioning (hypodysfibrinogenemia) of fibrinogen. The decreased fibrinogen concentration in congenital fibrinogen deficiency necessitates fibrinogen replacement therapy with fresh frozen plasma, cryoprecipitate, or human fibrinogen concentrate. However, the use of fresh frozen plasma and cryoprecipitate is limited owing to their longer transfusion time, requirement of high doses, volume overload, risk of viral transmission, and other safety concerns. The availability of human fibrinogen concentrate has made it the preferred replacement alternative due to its reduced risk of viral transmission, smaller infusion volume, and accurate dosing. The hemostatic efficacy and safety of human fibrinogen concentrate in congenital fibrinogen deficiency is well established in the literature. We review the prevalence of congenital fibrinogen deficiency in India and the current role of human fibrinogen concentrate in its management.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic malignancy with an aggressive behavior, seen commonly in the elderly age group and usually involves the skin. Evaluation, diagnosis, and treatment pose unique challenges with poor outcomes. We report a case of an 18-year-old female who presented with lymphadenopathy and bicytopenia. The diagnosis of BPDCN was an initial challenge. She thereafter received intensive chemotherapy followed by an allogenic partially mismatched sibling donor hematopoietic cell transplantation, only to relapse in a few months. Beginning from the diagnosis, the management of this rare disorder and its relapse until her last followup provided several learning opportunities.

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Primary lymphoma of the ovary are unusual. Malignant lymphoma of ovary are seen most commonly as a sequalae of disseminated nodal disease. We in this case report have discussed the case of primary diffuse large B-cell lymphoma with unilateral ovarian involvement in a 47-year-old woman which was diagnosed incidentally. Preoperative ultrasonic imaging showed multiple hemorrhagic follicles along left ovary with largest measuring 19x12mm. Total Hysterectomy with bilateral salpingo-ophorectomy was done. The present diagnosis of primary ovarian diffuse large B cell lymphoma of ovary was made after immunostaining.

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