ESIM: Winter School in Riga 2015 Case report Imanta Ozola Zālīte Pauls Stradins Clinical University Hospital Latvia 29.01.2015.
January, 2006 32 y., man 2-3 weeks fatigue fluidal stool 2 times per day weight loss 20 kg Psoriasis Smoking (20 pac years) BMI 17 kg/m 2 Arterial blood pressure 100/70 mmhg Heart rate 92 x Breathing rate 18 x Abdomen soft, painless CRP 32 mg/l [0 5.0 mg/l] Hb Er MCV Leu Platlets 103 g/l [130 170 g/l] 2.3*10 12 /l [4.5 6.00*10 12 /l] 130 fl [80 100 fl] 3.9*10 9 /l [4.0 10.0 *10 9 /l] 250*10 9 /l [150 400 *10 9 /l] Total protein 45 g/l [64 82 g/l] Albumin 31 g/l [35 50 g/l] Hidden blood in the stool - negative
January, 2006 Upper endoscopy Infiltrative gastric mucosa. Duodenum mucosa with scalloped, inflammated appearance. H. pylori test - negative Biopsies: Upper endoscopy Chronic active inflammation in the gastric mucosa, with incomplete erosive defects, sclerotic changes and mucosal atrophy. Massive clusters of eosinophils in the gastric mucosa. Colonoscopy Erosions in the terminal part of ileum, with fibrin and contact sangvination. Colonoscopy Crypt distortion, mikroabscesses, ulceration with diffuse lymphocyte, neutrophil infiltration. Crohn's disease. Abdominal CT Splenomegaly 15 x 4,7 cm. Mesenterial lymphadenopathy 1,2-1,8 cm.
January, 2007 BMI 21,4 kg/m 2 Abdominal CT, US still splenomegaly (16,7 x 5,6 cm), mesenterial lymphadenopathy February, 2008 Upper endoscopy In the gastric mucosa - 1mm large separated shallow defects with fibrin in the center. Inflammatory picture. Histology: Chronic, diffuse, active gastritis. Duodenal villous: crypt ratio 3: 1, intraepithelial lymphocytes - 40-41 lymphocytes per 100 epithelial cells. Morphological picture might meet celiac disease, Marsh I stage. Anti-gliadine antibodies IgA <1.2 [<12] IgG 1 [<12] PSA 0.29 [< 4 ng/ml] CEA 1.5 [< 3.4 ng/ml] CA 19-9 4.0 [< 39 U/mL ]
May, 2011 Colonoscopy: Throughout the length of colonic mucosa segmental damage with comprehensive small erosions. Terminal ileitis with partial ileo-caecal valve stenosis. Rtg-scopia, x-ray-graphia ventriculi et intestine: contrast arcade through the gastrointestinal tract slowed, but stenotic intestinal sections can not see. Lung CT: cylindrical bronchiectasis, lung right lower lobe pneumonia
September, 2012 Colonoscopy: In the middle part of Colon transversus ulcerose - scarred stricture with a residual diameter of 4 mm. Throughout the length of the colon superficial, minor erosion. Histology: Active chronic ulcers (fibrin, leukocytes, fibrotic stroma). RTG-scopia, Rtg-graphia ventriculi, intestini tenue et crassi: Local colonic stricture flexura coli sinistra area - characteristic malignoma. Distal part of ileum as Crohn`s disease. Diagnostic laparotomy: Could not see malignant stricture. Took biopsy from mesenterial lymphnode. Histology conclusion: Initials B cell lymphoproliferative infiltration (only the follicle). Marginal zone lymphoma. Onkocouncil decided that lymph node histological and radiological examinations comply with the Non-Hodgkin's lymphoma. Chemotherapy indicated. By lung and abdominal CT`s follow-up, chemotherapy with positive disease course.
2014 Progressive fatigue Fever, body temperature > 39 0 C Upper endoscopy Visually gastric mucosal atrophy picture. Histology: Chronic active gastritis and duodenitis Makrocytic anemia (Er 3.4 milj/l, Hb 101 g/l) Vit. B12 <83 [187 1059 pg/ml] HLA - B27 negative HIV ½, HIV 1Ag- negative Hidden blood in the stool not found Colonoscopy Ileocaecal area deformation. Colon distal some tiny, pale scars. Histology: Colonic mucosa architectonics moderately deformed. Colon mucosa crypts mostly normal structure.
2014 Lung CT: Multiple consolidates in the both lungs, more pronounced in left lungs lower lobe limfangoitis. Granulomatous lesions in both lungs? Repeated biopsy: Transabdominal US-guided needle biopsy of the mesenteric lymph nodes Histology: Multiple epiteloid cell granulomas, including the necroses and giant cells. Granulomatous lymphadenitis, needed more data on tuberculosis. TBC investigations negative (bronchial washing, sputum, stools, GeneXpert test in sputum, stool, bronchial washing)
? IgA <0.062 [0.7 4 g/l] IgG 0.8 [7 16 g/l] IgE 0.1 [0.1 0.4 mg/l]
Common variable immunodeficiency syndrome (CVIDS) Criteria? 1) Significantly reduced total serum concentrations of IgG 2) Low IgA and/or IgM 3) Poor or absent response to immunization 4) The absence of any other defined immunodeficiency state Clinical manifestations, epidemiology, and diagnosis of common variable immunodeficiency in adults, Ahn S. Cunningham-Rundles C, Aug 27, 2013, UptoDate
Manifestations? Recurrent infections Lung diseases (granulamotous - "granulomatous-lymphocytic interstitial lung disease"; obstructive or restrictive disease) Gastrointestinal disease (Inflammatory bowel-like disease, which may resemble ulcerative colitis, ulcerative proctitis, Crohn's-like disease, or microscopic colitis; Sprue-like illness with flat villi; Nodular lymphoid hyperplasia; Pernicious anemia; Bacterial overgrowth; Protein-losing enteropathy; Nonspecific malabsorption; Gastrointestinal lymphoma) Autoimmune disease (Autoimmune hemolytic anemia and immune thrombocytopenia, Rheumatoid arthritis Pernicious anemia, Autoimmune thyroiditis, Vitiligo Granulomatous infiltration Lymphadenopathy/splenomegaly Malignancies (Non-Hodgkin lymphomas, gastric cancers) Clinical manifestations, epidemiology, and diagnosis of common variable immunodeficiency in adults, Ahn S. Cunningham-Rundles C, Aug 27, 2013, UptoDate
What else, if not CVIDS? SECONDARY hypogammaglobulinemia => Decreased production Drugs (glucocorticoids, rituximab, anti-epileptics) Malignancy and pre-malignant disorders (chronic lymphocytic leukemia, lymphoma, multiple myeloma) Systemic illnesses causing bone marrow suppression Thymoma => Increased loss protein-losing enteropathies, such as intestinal lymphangiectasia, nephrotic syndrome, burns, traumas (loss of fluids) PRIMARY hypogammaglobulinemia (in adults) IgG subclass deficiencies Hyper IgM syndromes Combined immunodeficiency's Clinical manifestations, epidemiology, and diagnosis of common variable immunodeficiency in adults, Ahn S. Cunningham-Rundles C, Aug 27, 2013, UptoDate
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