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→Autopsy Findings
== Clinical Summary ===This 23-year-old black female delivered a stillborn infant by Caesarean sectionwent to her doctor because of chronic fatigue, bone pain, following which she experienced excessive uterine bleeding including the passage of blood clotsand easy bruising and frequent nose bleeds. Further study revealed an enlarged spleen Physical exam demonstrated hepatomegaly and splenomegaly and lab results demonstrated anemia and thrombocytopenia (platelet count 58,000). A splenectomy was performed with an uneventful postoperative course other than persistent pain in her right leg (the right hip had been fractured 2 years earlier). Subsequently she developed aseptic necrosis of the left femoral head requiring Biopsy and further workup led to a prosthetic replacement. Microscopic evaluation of the bone removed at the time diagnosis of surgery revealed Gaucher cells in the marrow spacedisease.
== Images ==
File:IPLab5Gaucher8.jpg|This is a higher-power photomicrograph of the spleen from this case. At this higher power individual cells can be better appreciated and the fibrillar nature of the eosinophilic cytoplasmic material can be seen.
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== Virtual Microscopy ==
<peir-vm>IPLab5Gaucher</peir-vm>
== Study Questions ==
The third pattern, type III, is usually somewhat intermediate between types I and II. These patients are usually juveniles and have systemic involvement like in type I but they have progressive central nervous system disease that usually begins in the second or third decade of life.</spoiler>
* <spoiler text="What type of Gaucher disease did this patient likely have?">This was an adult who had pancytopenia and aseptic necrosis of bone. This is consistent with type I. In type I symptoms and signs first appear in adult life and are related to splenomegaly or to bone involvement by Gaucher cells. These patients have pancytopenia or thrombocytopenia secondary to hypersplenism and pathologic fractures and bone pain due to expansion of the marrow space by Gaucher cells.</spoiler>
== Additional Resources ==
=== Reference ===
* [http://emedicine.medscape.com/article/944157-overview eMedicine Medical Library: Gaucher Disease]
* [http://www.merckmanuals.com/professional/hematology_and_oncology/spleen_disorders/splenomegaly.html Merck Manual: Splenomegaly]
* [http://www.merckmanuals.com/professional/pediatrics/inherited_disorders_of_metabolism/lysosomal_storage_disorders.html Merck Manual: Lysosomal Storage Disorders]
=== Journal Articles ===
* Barone R, Pavone V, Nigro F, Chabàs A, Fiumara A. [http://www.ncbi.nlm.nih.gov/pubmed/10792292 Extraordinary bone involvement in a gaucher disease type I patient]. ''Br J Haematol'' 2000 Mar;108(4):838-41.
=== Images ===
* [{{SERVER}}/library/index.php?/tags/1670-gaucher_cell PEIR Digital Library: Gaucher Cell Images]
* [http://library.med.utah.edu/WebPath/HEMEHTML/HEMEIDX.html Webpath: Hematopathology]
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[[Category: IPLab:Lab 5]]