Kai Rou Tey, MD, Kristen Barrett, MD, Richa Jain, MD, Thomas D

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Presentation transcript:

Vanishing Bile Duct Syndrome with Hemophagocytic Lymphohistiocytosis After Minimal Change Disease  Kai Rou Tey, MD, Kristen Barrett, MD, Richa Jain, MD, Thomas D. Boyer, MD, Archita Desai, MD  The American Journal of Medicine  Volume 129, Issue 12, Pages e315-e319 (December 2016) DOI: 10.1016/j.amjmed.2016.05.029 Copyright © 2016 Elsevier Inc. Terms and Conditions

Figure 1 Liver needle biopsy: ×200, hematoxylin-eosin (A) and ×400, Periodic acid–Schiff with diastase (B). The lobules show prominent cholestasis and Kupffer cell hyperplasia with mild inflammation. The American Journal of Medicine 2016 129, e315-e319DOI: (10.1016/j.amjmed.2016.05.029) Copyright © 2016 Elsevier Inc. Terms and Conditions

Figure 2 Liver needle biopsy: (A) ×400, hematoxylin-eosin: The portal tracts show the hepatic artery without an accompanying bile duct. There is prominent ductular reaction with neutrophilic infiltrate. The loss of bile ducts is highlighted by trichrome stain (B, ×400) and immunohistochemical stain (C, ×200) for cytokeratin 19 (×200, Clone A53-B/A2.26, Ventana, Roche, Tucson, AZ). The American Journal of Medicine 2016 129, e315-e319DOI: (10.1016/j.amjmed.2016.05.029) Copyright © 2016 Elsevier Inc. Terms and Conditions

Figure 3 Magnetic resonance cholangiopancreatography coronal view showing markedly enlarged spleen, measuring 21.5 cm (right) compared with prior imaging 4 months ago (left). The liver appeared normal in size and configuration, there is no intrahepatic or extrahepatic biliary ductal dilation, and the gallbladder is absent. The American Journal of Medicine 2016 129, e315-e319DOI: (10.1016/j.amjmed.2016.05.029) Copyright © 2016 Elsevier Inc. Terms and Conditions

Figure 4 Bone marrow biopsy showing hypocellular bone marrow, with prominent hemophagocytosis. There are virtually no myeloid progenitors. Noted: foamy cytoplasm of histocyte. The American Journal of Medicine 2016 129, e315-e319DOI: (10.1016/j.amjmed.2016.05.029) Copyright © 2016 Elsevier Inc. Terms and Conditions