Primary immunodeficiency diagnosed at autopsy: a case report

Rheumatoid Arthritis Complicated With Systemic A5myloidosis Diagnosed At Autopsy: A Case Report

Distribution of Primary Immunodeficiency Disorders Diagnosed in a Tertiary Referral Center, Tehran, Iran (2006-2013)

Background: Primary immunodeficiency disorders (PID) are a group of hereditary disorders characterized by an increased susceptibility to severe and recurrent infections, autoimmunity, lymphoproliferative disorders, and malignancy. Objective: To evaluate the demographic and clinical data of PID patients diagnosed in a referral pediatric hospital. Method: All PID cases with a confirmed diagnosis,...

Primary leptomeningeal melanocytosis--a case report with an autopsy diagnosis.

INTRODUCTION Primary melanocytosis of the leptomeninges is a rare tumor, most likely originating from the melanocytes in the leptomeninges. The average survival is only about 5 months. CASE REPORT A 61-years-old woman presented with headache, amaurosis and hallucinations lasted for two months, and she had been treated at the Clinic for Psychiatry and Clinic for Infectious Diseases. The cerebr...

Severe combined immunodeficiency presented with septic arthritis: A case report

Severe combined immunodeficiency is a rare, fatal syndrome of diverse genetic cause in which there is combined absence of T-lymphocyte and B-lymphocyte function (and in many cases also natural killer, or NK lymphocyte function). These defects lead to extreme susceptibility to serious infections. Without intervention, the T and B-cell dysfunction usually results in severe infection and death in ...

Hyper-IgM Immunodeficiency with Enamel Defects: a Case Report

Background: Hyper-Immunoglobulin M (IgM) syndrome (HIGM) is a rare primary immunodeficiency in which defective B-cell isotype switching results in a phenotype characterized by elevated or normal serum IgM levels and low levels of other Ig classes, leading to an increased susceptibility to infection, neutropenia, autoimmune disorders, and malignancies. In this disease, a mutation occurs in the C...