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Malignancy

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).

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Malignancy

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  1. 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)

  2. GI Diseases (n=248) Nodular lymphoid hyperplasia 10 Malabsorption, no other diag. 10 Giardiasis 8 UC 4 Ulc. Proctitis 3 Crohn’s disease 9 Malnutrition needing TPN 5 Campylobacter enteritis 5 Cunningham-Rundes and Bodian (Clin Immunol 1999; 92:34-48)

  3. 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

  4. 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

  5. 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

  6. Treatment • Early and aggressive antibiotic therapy, • Replacement with IVIG: 400 mg/kg/month • Ongoing therapy for autoimmune and inflammatory disorders & surveillance for malignancy

  7. 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, hypotensionanti-IgA antibodies (IgE isotype)

  8. IVIG • Receive very low IgA product is completely IgA deficient • Risk of infection : HIV : no risk HCV : more severe course in CVID patients

  9. 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

  10. Treatment Eur J Clin Invest 2000;30(3) :252-9 • Retinoic acidDecreased vitamin A level in CVID patients Associated with chronic bacterial infection and splenomegalySupplementation : IL-10 ,  TNF-ain vivo:  IgA ,  mitiogen response to PHA • Cimetidine ? • IL-10 ?

  11. Mortality and Survival • 248 patients; median follow-up 7 yrs (0-25) • 57 died from 1-32 yrs after diagnosis, ages 5-90 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)

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