Symptomatic Primary Selective IgM Immunodeficiency - B Lymphoid Cell Defect in Adult Man with Secondary HLH Syndrome.

Selective immunoglobulin M deficiency(sIgMD) is a rare form of dysgammaglobulinaemia characterized by an isolated low level of serum immunoglobulin M (IgM). It was an incidence of less than 0.03% in the general population and 1% in hospitalized patients. sIgMD may occur as a primary or secondary condition. sIgMD is much more common than primary .Hemophagocytic lymphohistiocytosis (HLH) is also a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes and can be primary or secondary, characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations. We report an adult case of primary sIgMD with absent B lymphoid cells and secondary HLH syndrome who presented with recurrent infections, fever and pancytopenia.

Life-Threatening Thrombo-embolic Events in a Case of Dengue Hemorrhagic Fever.

In Dengue Hemorrhagic Fever (DHF), hemorrhagic manifestations are common but thrombotic events are uncommonly reported, despite the wide range of increased procoagulant activity during Dengue Fever illness. We report a case of a 55-year-old man of Asian Indian ethnicity who developed large vein thrombotic event -Deep Vein Thrombosis (DVT) and Pulmonary Embolism (PE) in the acute phase of DHF. His condition was further complicated by associated thrombocytopenia. The etiological connections between dengue viral infection with thrombocytopenia, DVT/PE and abnormal thrombophilia profile as well as the treatment dilemmas posed in treating a patient of DF with hemorrhagic manifestations and associated DVT/PE, and the role of eltrombopag are discussed.

Near Fatal Poisoning by Isoniazid and Rifampicin.

Since six decades, Isoniazid and Rifampicin are used as first line drugs for treatment of tuberculosis. The minimum acute lethal or toxic dose of Rifampicin is not well established. However, non-fatal acute overdoses in adults have been reported with doses ranging from 9 to 12 gm and fatal acute overdoses with doses ranging from 14 to 60 gm. Isoniazid, if acutely ingested, even 1.5 to 2 gram may cause toxicity in adults. We report a case of Pott's spine on ATT, who took massive overdose of Rifampicin (>18 gm) and Isoniazid (>12 gm) and reported late (almost 36 hours) after ingestion. He was treated successfully with pyridoxine, hemodialysis and supportive care.