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Severe combined immunodeficiency with leukopenia (reticular dysgenesis) in siblings: immunologic and histopathologic findings.

The hematologic and histologic features of two, nontwin, male siblings with severe combined immunodeficiency and variable granulocytopenia are compared to the four previously reported cases of reticular dysgenesis. These sibs died at 50 and 3 days of age, respectively, with Pseudomonas sepsis and congenital cytomegalovirus infection, respectively. A maternal uncle has selective IgA deficiency. Cord blood from the second sib contained a normal percentage of E-rosetting lymphocytes; however, these lymphocytes failed to respond to mitogenic stimulation in vitro. Erythrocyte and lymphocyte levels of adenosine deaminase were elevated in the father and the second sib. Serum immunoglobulin concentrations were low in both siblings.

Adenosine Deaminase↗

Infectious complications of febrile leukopenia.

It can be foreseen that in the years to come major improvements in neutropenic host infections will be achieved regarding the exact identification of risk factors, allowing better patient stratification; the application of molecular techniques to recognize pathogens; the development of effective new oral antimicrobials allowing home therapy or abbreviated hospitalization; the development of new antifungals; and the development of new effective immunomodulators and cytokines to ameliorate chemotherapy-induced neutropenia. In the years to come the threat of nosocomial infections unfortunately will not be eliminated, while the development of major new parenteral antibiotics cannot be foreseen. It is therefore the caregiver/physician himself who, by applying rational antibiotic policies and strict handwashing rules, will probably escape, for his neutropenic patient's sake, the imminent threat of multiresistant pathogens.

Anti-Infective Agents↗

[Immuno-allergic thrombopenias and leukopenias induced by drugs].

Certain types of cytopenia are due to the destruction of blood cells by an antibody, active only in conjunction with a drug which has previously provoked sensitivity in patients when administered in standard doses. Drug-induced thrombocytopenia and leucopenia of allergic origin are relatively rare. They produce characteristic symptoms, i.e. brutal onset and acute development of the disease; healing takes place when the drug in question is withdrawn. Haematological diseases of drug-related aetiology are being brought to light by serological methods which detect the specific antibody for the drug responsible for the accident. The physiopathological mechanism is still not clearly elucidated.

Drug Hypersensitivity↗