hematopoietic system
• massive splenomegaly results from extensive expansion of the red pulp
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• extramedullary hematopoietic foci are found in the liver
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• severe hemolytic anemia with significantly decreased red blood cell count, hemoglobin and hematocrit
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• high numbers of large reticulocytes and small spherocytes, anisocytosis pholychromatophilia due to a high degree of reticulocytosis, poikilocytosis with many bizarrely shaped cells and fragments, increased red blood cell distribution width, and spherocytic red blood cells with membrane loss
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• approximately one third of normal red blood cell concentration at 6 weeks of age
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• approximately one thrid the normal level at 6 weeks of age
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• approximately one thrid the normal level of hemoglobin at 6 weeks of age
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anisocytosis
(
J:157766
)
immune system
• massive splenomegaly results from extensive expansion of the red pulp
|
homeostasis/metabolism
• approximately three times normal levels
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• plasma iron levels are significantly higher than normal
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• extensive iron accumulation in the kidney
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liver/biliary system
renal/urinary system
• extensive iron accumulation in the kidney
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growth/size/body
• massive splenomegaly results from extensive expansion of the red pulp
|