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      Atypical Fibroxanthoma


 

               

Acute pancreatic graft versus host disease (GVHD )may be noted in autopsies of children with congenital immune deficiencies with GVHD of other organs.  Visit: Pancreatic Pathology Online

Patients with pancreatic GVHD may have a history of  allogeneic bone marrow transplant, fetal liver or thymus transplant, or may have received nonirradiated blood products and had evidence of GVHD in other organs.

Careful attention to pancreatic ducts is necessary for diagnosis.

Microscopic features include lymphocytes around large to medium ducts, damage to ductal epithelium (focal necrosis, reactive nuclear changes, inspissated secretions in duct lumens), and periductal edema.

Immunoperoxidase stain for HLA-DR may show strong-to-moderate staining of duct epithelium.

                   

Pathology of the pancreas in severe combined immunodeficiency and DiGeorge syndrome: acute graft-versus-host disease and unusual viral infections. Hum Pathol. 1994 Sep;25(9):908-14.

Review of autopsies of 28 children with severe combined immunodeficiency (SCID) or combined immunodeficiency (CID) and three with DiGeorge syndrome showed a high incidence of acute graft-versus-host disease (GVHD) in the pancreas. Acute GVHD (seven cases: four SCID, two CID, and one DiGeorge syndrome) was characterized by lymphocytes around large to medium ducts, damage to ductal epithelium (focal necrosis, reactive nuclear changes, inspissated secretions in duct lumens), and periductal edema. Changes were judged indeterminate but suspicious for GVHD when ductal damage was slight (six cases: three SCID, two CID, and one DiGeorge syndrome). All patients with pancreatic GVHD had received allogeneic bone marrow, fetal liver or thymus transplant, or nonirradiated blood products and had evidence of GVHD in other organs. Immunoperoxidase stain for HLA-DR showed strong-to-moderate staining of duct epithelium in two of four GVHD cases for which blocks were available. This change was nonspecific; weaker staining for HLA-DR was seen in cases with nonspecific abnormalities and in viral pancreatitis. Four cases had histological evidence of viral infection: two had cytomegalovirus pancreatitis, one had patchy parenchymal necrosis caused by adenovirus, and one had giant cell pancreatitis caused by parainfluenza virus. Mild nonspecific changes, such as focal fat necrosis or acinar dilatation, were seen in seven cases. One case had unexplained marked pancreatic atrophy and fibrosis. Acute pancreatic GVHD is not uncommon in autopsies of children with congenital immune deficiencies with GVHD of other organs; however, this finding may not have strong clinical implications in this group of patients. Careful attention to pancreatic ducts is necessary for diagnosis. Unusual viral pancreatitis may also be seen in this group, as well as nonspecific abnormalities.

Anatomy of Normal Pancreas ;Normal Islets of Langerhans ; The Apud Concept ; An approach to reporting of pancreatic specimen ; Reporting of pancreatic biopsies for the diagnosis of neoplastic lesions ; Reporting of ampullary and periampullary biopsies for the diagnosis of neoplastic lesions ;Reporting of Pancreatico duodenectomy (Whipple's operation) specimen ; Reporting of Distal Pancreatectomy Specimen ; Developmental Defects of Pancreas ; Nesidioblastosis ; Pancreas Divisum ; Aberrant(Ectopic)Pancreas ; Annular Pancreas ; Pancreatic Agenesis ; Non-Neoplastic Pancreatic Cysts  ; Pancreatitis ; Acute Pancreatitis ; Chronic Pancreatitis ; Autoimmune Pancreatitis ; Herpes Simplex Pancreatitis ; Diabetes Mellitus ; Neoplasms of the Endocrine Tumours ; Islet Cell Tumours ; Glucagonomas  ; Insulinomas  ; Somatostatinoma ; VIPomas ; Pancreatic Polypeptide-Secreting Tumours ; Enterochromaffin Cell (Carcinoid) Tumours ; Pancreatic Gastrinoma ; Corticotropinoma ; Multiple Endocrine Neoplasia (MEN) Syndrome ; Carcinoma of the Pancreas ;Exocrine Pancreatic Tumours and Tumour -Like Lesions ; Paediatric Pancreatic Tumours ; Acinar cell carcinoma ; Pancreatoblastoma Mucinous Cystic Tumours.

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