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.
Kimura's disease is a chronic, benign inflammatory condition of the subcutaneous tissue. It presents as painless, subcutaneous nodules of the head and neck, which are firm, painless, and may be single or multiple. It is most commonly seen in young adult Asian men. The nodes remain stable or may slowly enlarge over time. Elevated serum immunoglobulin E (IgE) levels, peripheral blood eosinophilia and lymphoid proliferation with eosinophilic infiltration on histopathological examination are the characteristic features. Spontaneous regression is usually seen. The diagnosis of Kimura's disease can be difficult and misleading, and it is important not to ignore histopathological features.
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.
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.
Relapsed and refractory (RR) acute lymphoblastic leukemia (ALL) poses unique and difficult challenges to a practicing clinician in India where access to novel immunotherapies is limited. Between 2017 and 2020, eight patients with B-cell ALL at our center received inotuzumab ozogamicin (IO) monotherapy on compassionate access, as salvage therapy after at least two lines of conventional therapy failure, and most often as outpatient infusion. Eight patients (21–60 years, three females) received IO. Three patients had morphologic relapse and five patients reported persistent measurable residual disease (MRD). The best response on IO therapy achieved was negative MRD in six of seven patients and complete response (CR) with persistent MRD in one. One patient died (intracranial hemorrhage) before completion of first cycle. All responding patients were transplant eligible and four patients (57%) underwent allogeneic hematopoietic cell transplantation (Allo-HCT). Median follow-up of this cohort is 9 months (4–29.6 months), four patients (57%) are alive as stable with negative MRD. No significant infusion reactions occurred during therapy. Three patients developed grades III and IV neutropenia, two patients showed grade III transaminitis, and two patients developed post-HCT severe sinusoidal obstruction syndrome (SOS). IO is a feasible outpatient based salvage therapy to improve the remission status in RR B-cell ALL.
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