Budd-Chiari syndrome and extensive inferior vena cava thrombosis treated with sequential interventional radiology and transjugular intrahepatic portosystemic shunting: A case report from Kenya.

Budd-Chiari syndrome is a rare disease characterized by the obstruction of hepatic venous outflow. Stepwise treatment options aimed to relieve obstruction and prevent complications of Budd-Chiari syndrome are medical therapy, interventional recanalization, and surgery. Aggressive interventions for complicated Budd-Chiari syndrome are placement of a transjugular intrahepatic portosystemic shunt, surgical shunting, or liver transplantation. Although literature suggests differences in the presentation and management between Europe and Asia, cases documenting successful use of stepwise management of Budd-Chiari syndrome in Sub-Saharan Africa are scarce. A 47-year-old male on treatment for chronic hepatitis B presented with abdominal pain and distension for 2 weeks and findings of gross ascites without stigmata of chronic liver disease. Laboratory investigations performed showed anemia, elevated transaminases, coagulopathy, and renal dysfunction. Abdominal ultrasound and computed tomography abdominal scan revealed filling defects in intrahepatic veins and inferior vena cava extending to bilateral renal and external iliac veins. Extensive workup for thrombophilia and myeloproliferative disorders was negative. The diagnosis was hepatic dysfunction secondary to inferior vena cava obstruction due to a thrombus in the setting of extensive inferior vena cava thrombosis, and heparin was initiated. However, due to lack of recanalization with anticoagulation, we performed aspiration thrombectomy, balloon angioplasty, and local thrombolysis. Transjugular intrahepatic portosystemic shunt procedure was subsequently done due to hepatic venous congestion and refractory ascites. He was discharged on oral anticoagulation. Imaging exams performed 4 months later showed patent inferior vena cava and transjugular intrahepatic portosystemic shunt, good flows in the portal vein and resolution of ascites.

Immunophenotypic expression profile of multiple myeloma cases at a tertiary hospital in Nairobi Kenya.

Introduction: Multiple myeloma (MM) is a plasma cell neoplasm that constitutes 10-15% of all hematopoietic neoplasms. Kenya is placed among the top five African countries for MM incidence and MM-related mortality. Prior studies have suggested that the aberrant expression of Cyclin D1, CD56, CD117 and Ki-67 on neoplastic plasma cells is useful in disease prognostication. The prevalence and significance of expression of these markers in a cohort of MM cases in Kenya has not been studied previously. Methods: A retrospective cross-sectional study was carried out at the Aga Khan University Hospital, Nairobi. The study population included 83 MM cases with available trephine blocks archived between 1st of January 2009 and 31st of March 2020. Immunohistochemical expression of Cyclin D1, CD56, CD117, and Ki-67 was analyzed and scored. The biomarkers were described using frequencies based on the positive and negative results. Fisher's exact test was used to determine the association between the immunophenotypic markers and categorical variables. Results: Of the 83 selected cases, expression of Cyclin D1, CD56, CD117 and Ki-67 was identified in 28.9, 34.9, 7.2, and 50.6%, respectively. Cyclin D1 positivity was significantly associated with hypercalcemia. Absence of CD117 expression was noted to be associated with adverse risk parameters including an IgA isotype or light chain disease, International Staging System (ISS) stage III disease, abnormal baseline serum free light chains (sFLC) and a high plasma cell burden. Conclusion: Cyclin D1 expression was congruent with previously reported studies. The frequency of CD56 and CD117 expression was lower than previously reported. This may be due to differences in disease biology between the study populations. Approximately half of cases were Ki-67 positive. Our data showed limited associations between the expression of studied markers and clinicopathologic variables. However, this could be attributed to the small study sample size. We would recommend further characterization of the disease in a larger prospective study with the inclusion of survival outcomes and cytogenetic studies.

Adult secondary hemophagocytic lymphohistiocytosis.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of excessive inflammation and tissue destruction due to abnormal immune activation. HLH carries a very high mortality, and while delays in patients' presentation to hospital, time to suspicion of HLH, investigation, and initiation of therapy all play a part, mortality remains high even with timely diagnosis and treatment. Classical manifestations of HLH include persistent fever, cytopenias, and liver dysfunction. Case presentation: We present four cases of secondary HLH, highlighting the demographic and clinical characteristics of these patients, underlying triggers (including systemic lupus erythematosus, lymphoproliferative disorders, and leishmaniasis), together with challenges associated with the diagnosis and treatment of this rare disorder and a brief review of literature. Conclusion: HLH has protean manifestations and requires a high index of suspicion as it can be a great clinical masquerader. Mortality due to multiorgan failure is often high even with early recognition and treatment.