1 / 12

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).

joann
Télécharger la présentation

Malignancy

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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)

More Related