Post on 25-Dec-2015
NYU Medicine Grand Rounds Clinical Vignette
Benjamin P. Geisler, MD, MPHPGY-2
2/5/2014
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
•59 year old woman with epigastric pain for four days
Chief Complaint
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
•For the past 4 days, has had multiple episodes of non-bloody, non-bilious vomiting
•Thirteen days prior had stay in observation unit for diarrhea and dehydration, empirically started on metronidazole
•Intense, sharp, crampy right foot pain for three days after walking half a block, relieved by rest
History of Present Illness
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
•Previous workup for celiac disease:•Transglutaminase IgA (92 [normally <4] U/ml)•Esophagogastroduodenoscopy 4/2013 (↑ intraepithelial lymphocytes, mild-moderate villous atrophy)•Capsule endoscopy 5/2013 consistent with active Celiac disease in the small bowel
•Improved with gluten-free diet; transglutaminase IgA decreased to 30 U/ml
History of Present Illness (continued)
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
Additional History
•Past Medical History:•Celiac Disease•Osteoporosis•Osteoarthritis
•Past Surgical History:•Status post total right hip arthroplasty 2010
•Social History:•From Ireland•Denis toxic habits
•Family History:•Celiac Disease in multiple relatives•Father died from unknown lymphoma at age 42•Brother with diabetes and myocardial infarction at age 51
Additional History (continued)
•Allergies: •Aspartame – urticaria
•Medications:•Metronidazole 500 mg three times per day•Bismuth subsalicylate 524 mg every hour as needed•Ibuprofen-diphenhydramine 600/114 mg nightly•Ergocalciferol 50,000 once per week•Folic acid 1 mg daily
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
Physical Examination
•General: anxious-appearing, pale, in painful distress•Vital Signs: T:97.8°F BP:176/92 HR:71 RR:20 and O2 sat:100% on room air•Epigastric tenderness•Right first toe cyanotic, sluggish capillary refill on right lower extremity without palpable dorsalis pedis pulse•Remainder of Physical Exam was normal
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
Laboratory Findings
•CBC: white cell count 16,500/µl (82% neutrophils, 14% lymphocytes), platelets 756,000/µl
•Remainder of CBC was within normal limits•Basic Metabolic panel: CO2 21 mmol/L, BUN 9 mg/dL
•Remainder of basic was within normal limits•Hepatic panel: alkaline phosphatase 147 U/L, albumin 3.1 g/dL
•Remainder of hepatic panel was within normal limits
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
Other Studies
•Arterial duplex: mild femoral-popliteal and mild to moderate tibio-peroneal occlusive disease
•CT abdomen/pelvis: diffuse calcific atherosclerosis, unchanged right adnexal cyst, scattered mesenteric lymph nodes without intestinal inflammation or obstruction, new non-occlusive 1.9 cm thrombus in the infrarenal aorta
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
• Infrarenal aortic thrombus of unclear etiology– Myeloproliferative disorder
• Essential thrombocytemia• Polycytemia vera• Primary myelofibrosis• Chronic leukocytemic leukemia
– Reactive thrombocytosis, e.g., from refractory celiac disease or lymphoma
Working or Differential Diagnosis
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
• Hospital Day 1-2:– Heparin and aspirin started– Peripheral blood sent for JAK2 (V617F)
mutation and t(9;22) translocation with BCR-ABL fusion; both subsequently returned negative
Hospital Course
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
• Hospital Day 3-6:– Open aortic thrombectomy, aortoplasty, and
mesenteric lymph node biopsy– Hydroxyurea started for persistent
thrombocytosis
Hospital Course (continued)
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
• Hospital Day 16:– Colonoscopy: mildly congested mucosa
throughout; nodular ileal mucosa; congested, friable fold in the ascending and adenomatous-appearing fold in the descending colon; hyperemic rectum
– Small bowel enteroscopy: diffuse scalloped and friable mucosa in the proximal and mid-jejunum without nodules, lesions, or masses
Hospital Course (continued)
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
• Hospital Day 20:– Biopsies with abnormalities in mesenteric
lymph node and small bowel• Slight excess of B-cells, felt non-diagnostic of
lymphoma• Increased number of γδ-T cells especially in ileum
with presence of large cells• Molecularly and immunopheno typically unclear
– Corticosteroids started
Hospital Course (continued)
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS
• Duodenum and jejunum:– Partial villous atrophy, crypt hyperplasia, moderate
inflammation, and patchy intraepithelial lymphocytosis
• Terminal ileum, ascending/left/sigmoid/rectum:– Lymphocytic infiltrate of intermediate to large, atypical,
pleomorphic CD3+, CD4(subset)+, CD5-, CD30(subset)+ cells
– Kappa-restricted HLA-DR+, CD5-, CD19+, CD20dim, CD30- CD79a+, CD103- clone
• Bone marrow:– Positive for T-cell receptor-beta gene rearrangement
Pathological Re-evaluation
UNITED STATES
DEPARTMENT OF VETERANS
AFFAIRS