Intravenous immunoglobulin death

IMMUNODEFICIENCY WITH HYPER-IGM (HIGMl), AN IMMUNOGLOBULIN ISOTYPE SWITCH DEFECT CHARACTERIZED BY ELEVATED CONCENTRATIONS OF SERUM IGM AND DECREASED AMOUNTS OF ALL OTHER ISOTYPES. AFFECTED MALES PRESENT AT AN EARLY AGE (USUALLY WITHIN THE FIRST YEAR OF LIFE) RECURRENT BACTERIAL AND OPPORTUNISTIC INFECTIONS, INCLUDING PNEUMOCYSTIS CARINII PNEUMONIA AND INTRACTABLE DIARRHEA DUE TO CRYPTOSPORIDIUM INFECTION. DESPITE SUBSTITUTION TREATMENT WITH INTRAVENOUS IMMUNOGLOBULIN, THE OVERALL PROGNOSIS IS RATHER POOR, WITH A DEATH RATE OF ABOUT 10% BEFORE ADOLESCENCE. 

Whereas idiopathic OM often resolves, spontaneously or after administration of intravenous immunoglobuline or corticosteroids [58], the outcome of paraneoplastic OM is more variable and depends on the tumor response to therapy. Children with paraneoplastic OM frequently respond to chemotherapy, adrenocorticotropic hormone, or immunomodulation [223], The response to immune therapy in adults with paraneoplastic OM is very modest, but prompt tumor therapy, immunomodulation, and depletion of IgG may be of some benefit [62, 224], Without antineoplastic therapy, the prognosis is usually poor, and symptoms often progress, ultimately causing death [62],... 

Infection is the primary cause of death in patients with chronic GVHD, and antimicrobial prophylaxis is an important component of the care of patients being treated for chronic GVHD. Patients should receive oral trimethoprim-sulfamethoxazole, penicillin, and acyclovir to prevent those infections commonly seen in immunocompromised patients. Some centers will also administer intravenous immunoglobulin to patients with low serum immunoglobulin G levels. 

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