Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc
Journal website http://www.thejh.org

Case Report

Volume 4, Number 3, September 2015, pages 193-195

An Atypical Case of Atypical Hemolytic Uremic Syndrome: Predominant Gastrointestinal Involvement, Intact Renal Function, and C5b-9 Deposition in Colon and Skin

Figure

Figure 1.
Figure 1. Biopsy of skin and colon. (A-C) Biopsy of non-lesional skin shows endothelial cell detachment with edema of the vessel wall, and concomitant vascular basement membrane zone reduplication (A). A venule contains a small intraluminal thrombus comprised of fibrin and platelets (B) (H&E). Immuohistochemical studies show prominent deposits of C5b-9 within the vessel walls (C) (iaminobenzidene). (D-E) Sections of the colon which demonstrate ischemic ulceration associated with vascular thrombosis (D) (H&E). C5b-9 deposits are prominent within the microvasculature of the colonic mucosa (E) (diaminobenzidene).

Table

Table 1. Laboratory Findings Pre- and Post-Treatment With Eculizumab
 
Initial LabsWeek 4*Week 10*
*Laboratory findings at weeks 4 and 10 post-reinduction with eculizumab. +Direct antiglobulin test.
Hemoglobin (g/dL)9.89.211
Platelets (× 103/μL)111368289
LDH (U/L)1,469217148
Haptoglobin (mg/dL)< 10372284.2
Serum creatinine (mg/dL)1.020.370.54
Estimated GFR53> 60> 60
Lipase (U/L)12441
Total Bilirubin (mg/dL)6.70.7
DAT+Negative
ADAMTS-13 activity (%)75%