malignant histiocytosis (mh) is a rare hematologic malignancy, especially in the first decade of life. the disease is clinically characterized by fever, hepatosplenomegaly, lymphadenopathy, pancytopenia and jaundice, and histologically by systemic proliferation of malignant histiocytes and hemophagocytosis. the prognosis is poor and often the diagnosis is not made before death. because of the r...

A 12-year-old male child reported with history of fever for last seven years. Hepatosplenomegaly, hepatic and bone marrow granulomas were the main features. Idiopathic Granulomatous Hepatitis (IGH), a rare syndrome amenable to immunosuppressive therapy was diagnosed.

In this case report we describe the first case of hemophagocytic lymphohistiocytosis with concurrent cutis marmorata telangiectatica congenita. She had pancytopenia and hepatosplenomegaly, hemophagocytic cells in spleen necropsy, and she died with respiratory failure and pseudomonas induced septicemia.

An unusually severe hypersensitivity reaction to carbamazepine is described, in which the patient presented with lymphadenopathy, hepatosplenomegaly, bone marrow suppression, immunosuppression and other features strongly suggestive of a lymphoma.

Wolman disease is a very rare autosomal recessive genetic disorder. The patients have the typical clinical finding of hepatosplenomegaly but with an abnormal lipid profile high levels total cholesterol (TC), triglycerides, and low-density lipoprotein (LDL-C), low level high-density (HDL-C). We report 1-month-old boy who had atypical TC level, both LDL-C HDL-C. study revealed compound heterozygo...

Summary Hepatosplenic γ/δ T-cell lymphoma (HSTL) is a very rare, aggressive extranodal affecting mainly young adults. Clinically, presents with symptomatic hepatosplenomegaly and systemic symptoms but without lymphadenopathy. The diagnosis confirmed after careful evaluation of bone marrow liver biopsies or, in some cases, diagnostic splenectomy. Overall, survival short regardless chemotherapy r...