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

• Enteropathy-associated T-cell lymphoma• Possible concurrent B-cell lymphoproliferative

disorder, likely low-grade B-cell lymphoma

Final Diagnosis

UNITED STATES

DEPARTMENT OF VETERANS

AFFAIRS