A case of fatal Epstein-Barr virus (EBV) associated lymphoproliferative disorder is reported in an 11 month old female. Heavy infiltrates of CD20 + and EBV EBER mRNA expressing lymphoid blasts were found to cause a series of ulcers along the entire length of the gastrointestinal tract and there was an ileal perforation. Similar infiltrates were also found in lymph nodes, spleen, and liver. Alth...

Follicular dendritic cells (FDCs) are unique immune system cells that contribute to the maintenance of humoral (i.e. antibody) immune responses. These cells are located in the follicles of secondary lymphoid tissues (e.g. lymph nodes and spleen), where they trap and retain antigens in the form of highly immunogenic immune complexes (ICs). FDC-ICs are composed of antigen plus specific antibody a...

Scabies, infection with Sarcoptes scabiei, is known to be predisposed to by poor body hygiene, environmental exposure, and systemic immunodeficiency. We report the case of an 83-year-old man with Sezary's syndrome who developed scabies limited to the skin of the upper chest, the same location where he had previously received electron beam radiation treatments for cutaneous T-cell lymphoma. Hist...

Tuberculosis remains a serious threat to public health, especially in sub-Saharan Africa. To determine the host and environmental factors responsible for tuberculosis in African households, the authors performed a prospective cohort study of 1,206 household contacts of 302 index cases with tuberculosis enrolled in Uganda between 1995 and 1999. All contacts were systematically evaluated for acti...

A 40-year-old man reported a 5-day history of fever and malaise, followed by a pruritic generalized rash. He had well-demarcated erythematous papules and plaques with scaling. The patient was diagnosed with secondary syphilis. The skin biopsy showed a psoriasiform lichenoid dermatitis with plasma cells. The anti-T. pallidum antibody confirmed the presence of spirochetes. He was also found to be...

Ataxia-telangiectasia (A-T) is a human autosomal recessive disease caused by mutations in the gene encoding ataxia-telangiectasia mutated (ATM). A-T is characterized by progressive cerebellar degeneration, variable immunodeficiency, and a high incidence of leukemia and lymphoma. Recurrent sino-pulmonary infections secondary to immunodeficiency and hematopoietic malignancies are major causes of ...

Primary immunodeficiency diseases (PID) are congenital disorders secondary to an impaired immune response. Infections, autoimmune disorders, atopy, and lymphoproliferative syndromes are commonly associated with this disorder. OBJECTIVE To present and discuss 3 infants diagnosed with PID. CLINICAL CASES The cases are presented of three patients with PID diagnosed during their first admission...

Activated PI3K-δ syndrome refers to a recently described primary immunodeficiency syndrome consisting of recurrent sinopulmonary infections, lymphadenopathy, mucosal lymphoid aggregates, increased susceptibility to Epstein-Barr virus and cytomegalovirus, and increased incidence of B-cell lymphomas. Variants in PIK3CD, which encodes the phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic su...

Impairment of any of the major components of the immune system (T-cells, B-cells phagocytes, complement) may result in clinical immunodeficiency. Immune defects can arise from intrinsic or heritable defects of lymphoid elements, failure of normal cellular differentiation, viral infection or other acquired causes. Clinical impairment of immunity is expressed as a marked susceptibility to opportu...

The recently described classical case of dyskeratosis congenita (DC)(1) lacked information as to whether the patient had features of immunodeficiency. The immune abnormalities described in X-linked DC include hypogammaglobulinemia (low IgM levels), severe B cell lymphopenia, low numbers of T cells, increased rate of apoptosis and marked reduction in cellular proliferation in short-term cultures...