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Malignancy NHL 7.7% - mostly extranodal, all B cell type Others – Waldenstrom’s macroglobulinemia – Hodgkin’s disease – Adenocarcinoma - stomach, ovary, colon – Sq cell Ca - vagina, skin, mouth, lung – Ca - breast, prostate – Melanoma Cunningham-Rundes and Bodian (Clin Immunol 1999; 92:34-48) GI Diseases (n=248) Nodular lymphoid hyperplasia Malabsorption, no other diag. Giardiasis UC Ulc. Proctitis Crohn’s disease Malnutrition needing TPN Campylobacter enteritis 10 10 8 4 3 9 5 5 Cunningham-Rundes and Bodian (Clin Immunol 1999; 92:34-48) Granulomatous disease 20% patients Lung, lymph nodes, skin, bone marrow, and liver Histology: non-caseating granuloma, indistinguishable from sarcoidosis Asymptomatic/ dyspnea if lung involvement/anemia or thrombocytopenia if hypersplenism Corticosteroid : effectively in some but increased risk of infection Splenectomy Laboratory Abnormalities IgG, mostly with IgA and IgM Lymphopenia 20% B cell numbers - mostly normal CD4/CD8 ratios, in CD4+CD45+ T cells Low or absent isohemagglutinin titers and specific Ab levels T cell in vitro proliferation : subnormal in 50% to mitogen, antigens Differential diagnosis Primary immunodeficiency syndrome X-linked agammaglobulinemia Transient hypogammaglobulinemia of infancy Infectious agents CMV, EBV, HIV Malignancy Lymphoma, multiple myeloma, CLL Protein-losing states Nephrotic syndrome, Protein-losing enteropathy Drugs Cyclophosphamide, phenytoin, Gold, Penicillamine XLA Mutation of gene Btk CVID -- -- -- -- Family Hx X-linked Onset Late 1st year Any age Recovery - THI - 1st year 2-4 y/o Lymph node small hyperplasia B cell umber absence near normal normal T cell normal subtle normal IgG ↓ IgA ↓ IgM ↓ IgG ↓ IgA ↓ IgM ↓ IgG ↓ IgA ↓ IgM or normal Ig -- Treatment Early and aggressive antibiotic therapy, Replacement with IVIG: 400 mg/kg/month Ongoing therapy for autoimmune and inflammatory disorders & surveillance for malignancy IVIG Trough levels > 350 mg/dL without infection Levels > 500 mg/dL if severe infection persists Back or abdominal pain, nausea, vomiting, chills, fever, and myalgia -> nonanaphylactic binding of infused Abs to microbial Ag True anaphylactic reactions : rare flushing, facial swelling, dyspnea, hypotension anti-IgA antibodies (IgE isotype) IVIG Receive very low IgA product is completely IgA deficient Risk of infection : HIV : no risk HCV : more severe course in CVID patients Treatment IL-2 Clin Immunol 2001;100(2) :181-90 15 patients received PEG-IL-2 12-18 mo / 29 control T cell proliferative response to mitogen T cell proliferative response to antigen (candida and tetanus) Antibody response to immunization with neoantigen bacteriophage fX 174 Days of bronchitis, diarrhea and joint pain Treatment Retinoic acid Decreased vitamin A level in CVID patients Associated with chronic bacterial infection and splenomegaly Supplementation : IL-10 , TNF-a in vivo: IgA , mitiogen response to PHA Eur J Clin Invest 2000;30(3) :252-9 Cimetidine ? IL-10 ? Mortality and Survival 248 patients; median follow-up 7 yrs (0-25) 57 died from 1-32 yrs after diagnosis, ages 590 yrs (median age 43) - 27% Causes -Lymphoma, cor pulmonale, hepatitis, malnutrition, other malignancies, vasculitis, etc. Poor prognostic signs: % of peripheral B cells, initial IgG level For each % in B cell numbers, risk of death on follow-up by a factor of 0.92 Cunningham-Rundes and Bodian (Clin Immunol 1999; 92:34-48)