Clinical Case. Dr. Akif BAYYİĞİT Dr. İlker Nihat ÖKTEN Dr. Serkan DUMANLI

Transcription

1 Clinical Case Dr. Akif BAYYİĞİT Dr. İlker Nihat ÖKTEN Dr. Serkan DUMANLI

2 Case: Mr. A.D. 44 Male Abdominal pain and fever Past Medical History: Facial paralyses 20 years ago Splenomegaly? Family History: Mother DM HT father lung cancer died at 57 Habits: Smoker 25 packet/year 2

3 Physical Exam (E.R.) Item General Vital Signs CVS Respiratory Abdominal Extremities Skin Physical Exam No acute distress BP 100/70, Hr 109 (NSR) fever:37,8 Oxygen saturation:96% on room air s1 + s2 + no murmur No wheezes, no crackles Diffuse discomfort and tenderness in all quadrants no defans no rebaund shifting dullness with percussion(suspicious) No abnormality No obvious skin changes 3

4 E.R. Evaluation Lab Values Wbc: neu:16430 bas:240 lym:850 Rbc: 6160 Hgb:11.2 Mcv:62,3 Mch:18,2 Plt: Ast: 28 Alt:24 ggt:62 ldh:273 amilaz:23 albumin:3,34 total bil:2,19 direct bil:0,41 ind. Bil:1,78 pt(ınr): 1,4 aptt:32,7 4

5 E.R. Evaluation Abdomen USG No patholgy in solid organs Increase in spleen size and subcapsular 31x21 mm sized heterogeneous hypoechoic area concordant with infarction Portal vein, inferior mesenteric vein, superior mesenteric vein and splenic arter not well visualized(thrombus?) Right low quadrant periceacal broad inflammatory changes and free fluid in the peritoneal space Abdominal tomography advisory 5

6 E.R. Evaluation Abdominal CT angiography Diffuse oesaphageal wall thickening Normal liver size and parenchyma no solid or cystic mass Normal pancreas and parenchyma Normal kidney shape and size Portal vein, inferior mesenteric vein, superior mesenteric vein and splenic arter not well visualized Spleen size has expanded to 185*96 mm and contours are lobulated.calcification visualised in the capsule. Diffuse gastric wall thickening 6

7 E.R. Evaluation Abdominal CT angiography Normal abdominal aorta and vena cava inferior Free fluid in all quadrants in the peritoneal cavity Wall thickening in ileocecal junction and sigmoid colon Diffuse collateral vasculer structures in the mesenterium Patchy wall thickening in colon and ileum that may be related to venous ischemia Diffuse vascularization increment in mesenteric lipoid structures and omental cake appearance Consultation with general surgeon: no acute abdomen 7

8 Hospitalization (Summary) 44 male, no known chronic illness Abdominal pain and fewer Acute abdomen (-) Ascites (+) splenomegali (+) Abdominal venous and arterial thrombus (+) (chronic) Hypocromic microcytic anemia (+) Lokocytosis (+) 8

9 DD Inherited Thrombophilia Factor V Leiden mutation Prothrombin gene mutation Protein S deficiency Protein C deficiency Antithrombin deficiency Rare disorders Dysfibrinogenemia Acquired Disorders Malignancy Presence of central venous catheter Surgery esp. orthopedic 9

10 DD Acquired Disorders Trauma Pregnancy Oral contraceptives Hormone replacement therapy Tamoxifen, thalidomide, lenalidomide İmmobilization Congestive failure Antiphospholid antibody syndrome Myeloproliferative disorders PV, ET Paroxysmal nocturnal hemoglobinuria İnflammatory bowel disease Nephrotic syndrome Behçet s disease 10

11 Laboratory Wbc Rbc 6160 Plt neu baso 240 lymp 850 hgb 11,2 mcv 62,3 mch 18,2 TSH 1,2 ft4 0,89 AST 28 ALT 20 GGT 62 LDH 266 D.BİL 0,4 İnd.BİL 1,21 AMİLAZ 60 PT-INR 1,4 TOTAL PRT 6,48 ALBUMİN 3,36 ALP 100 UREA 26 CREATININ 0,84 NA 137 K 4,1 İron 17 SIBC 242 Ferritin 12 Transferrin sat% 7 Vit B Folate 7 Smear: Microcytic hypochromic anemia, no atypic cell, ne precurser cell 11

12 Laboratory Antids-DNA HBs Ag ANA Anti-hbs Lupus anticoagulant anti-cardiolipin antibody anti beta 2 glicoprotein1 Anti-HIV Anti-HCV Alpha 1 antitripsin seruloplasmin Negative anti-mitochondrial antibody anti-liverkidney antibody anti-smooth muscle antibody Protein C protein S activity Factor V leiden gene mut prothrombin gene mut

13 Serum homocystein Patergy test bcr-abl test Jak-2 gen mut normal POSITIVE Gastroscopy: Grade 2-3 oesophageal varices in the mid-distal portion, 3-4 mm diamater ulcerous lesion in antrum Colonoscopy: Rectal varicose vein, normal colonic wall and normal terminal ileum 13

14 Pet-CT&Bone Marrow Biopsy Pet-CT: findings correlated with lymphoprolypherative disease or leukemia. Evaluation with bone marrow biopsy is advisory Bone Marrow Biopsy: polycytemia vera and primary myelofibrosis are included for differantial diagnosis. Although the lack of leucocytosis and thrombocytosis and the existence of erihyroid hyperplasia are signs against the diagnosis; the existence of dysplasia in the megakaryocytic series is accordant with early period primary myelofibrosis. 14

15 Treatment Low-Molecular Weight Heparin Spironolactone Propranolol Hydroxyurea 15

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17 Management

18 Management

19 Management