Difference between revisions of "IPLab:Lab 5:Gaucher Disease"

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== Clinical Summary ===
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== Clinical Summary ==
 
This 23-year-old black female delivered a stillborn infant by Caesarean section, following which she experienced excessive uterine bleeding including the passage of blood clots. Further study revealed an enlarged spleen 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 a prosthetic replacement. Microscopic evaluation of the bone removed at the time of surgery revealed Gaucher cells in the marrow space.
 
This 23-year-old black female delivered a stillborn infant by Caesarean section, following which she experienced excessive uterine bleeding including the passage of blood clots. Further study revealed an enlarged spleen 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 a prosthetic replacement. Microscopic evaluation of the bone removed at the time of surgery revealed Gaucher cells in the marrow space.
  
 
== Autopsy Findings ==
 
== Autopsy Findings ==
The surgical specimen was a 935-gram spleen. Its surface was pale with an area of bluish discoloration. The cut surface revealed the same pale appearance.  
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The surgical specimen was a 935 gram spleen. Its surface was pale with an area of bluish discoloration. The cut surface revealed the same pale appearance.  
  
 
== Images ==
 
== Images ==
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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.  
 
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.  
 
</gallery>
 
</gallery>
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== Virtual Microscopy ==
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<peir-vm>IPLab5Gaucher</peir-vm>
  
 
== Study Questions ==
 
== Study Questions ==
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* [http://emedicine.medscape.com/article/944157-overview eMedicine Medical Library: Gaucher Disease]
 
* [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/hematology_and_oncology/spleen_disorders/splenomegaly.html Merck Manual: Splenomegaly]
* [www.merckmanuals.com/professional/pediatrics/inherited_disorders_of_metabolism/lysosomal_storage_disorders.html Merck Manual: Lysosomal Storage Disorders]
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* [http://www.merckmanuals.com/professional/pediatrics/inherited_disorders_of_metabolism/lysosomal_storage_disorders.html Merck Manual: Lysosomal Storage Disorders]
  
 
=== Journal Articles ===
 
=== Journal Articles ===
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=== Images ===
 
=== Images ===
* [http://peir.path.uab.edu/library/index.php?/tags/1670-gaucher_cell PEIR Digital Library: Gaucher Cell Images]
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* [{{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]
 
* [http://library.med.utah.edu/WebPath/HEMEHTML/HEMEIDX.html Webpath: Hematopathology]
  

Latest revision as of 16:15, 3 January 2014

Clinical Summary[edit]

This 23-year-old black female delivered a stillborn infant by Caesarean section, following which she experienced excessive uterine bleeding including the passage of blood clots. Further study revealed an enlarged spleen 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 a prosthetic replacement. Microscopic evaluation of the bone removed at the time of surgery revealed Gaucher cells in the marrow space.

Autopsy Findings[edit]

The surgical specimen was a 935 gram spleen. Its surface was pale with an area of bluish discoloration. The cut surface revealed the same pale appearance.

Images[edit]

Virtual Microscopy[edit]

Study Questions[edit]


Additional Resources[edit]

Reference[edit]

Journal Articles[edit]

Images[edit]

The normal platelet count is 150,000 to 400,000/mm³.

Trauma (from fracture or surgery) is one cause of asceptic (or avascular) necrosis of bone, which is defined as the death of bone and bone marrow in the abscence of an infectious agent.

The normal spleen weighs 155 grams.