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Friday, April 4, 2014

Hyposplenism

         Absent or reduced splenic function, usually due to surgical removal, congenital aplasia, tumor replacement, or splenic vascular accident.
         Red blood cell abnormalities, including the presence of inclusions, nucleated red blood cells and target cells are commonly present. Persistent thrombocytosis with increased risk of thrombosis. Patients with hyposplenism are at increased risk of bacterial sepsis, especially due to infection by Pneumococci.
Conditions associated with hyposplenism:
1-    Sickle cell anemia
2-    Gluten induced enteropathy (Coeliac disease).
3-    Crohin's Disease and Ulcerative colitis
4-    Myelofibrosis and essential thrombocytosis
5-    SLE and RA
6-    Lymphoma and Multiple Myeloma
7-    Splenic irradiation and emolization
Diagnosis:
Determined by anatomic presence or absence of the organ, its size, and any lesions.
Function can be assessed by
Radiologic Techniques: X-ray, ultrasound, tomography, MRI, radionucleotide scanning
Morphologically: Peripheral blood smear- presence of Howell-Jolly bodies.
Complications:
Lifelong risk for Overwhelming Postsplenectomy infection (OPSI) Caused by Streptococcus pneumoniae and gram negative bacteria
Initial Symptoms: fever, chills, muscle aches, headache, vomiting, diarrhea, and abdominal pain
Progressive symptoms: bacteremic septic shock, extremity gangrene, convulsions, and coma
Mortality rate of 50-80% from onset of initial symptoms, 68% of those deaths occur within 24 hours and 80% occur within 48 hours
Prevention: routine vaccinations and prophylactic antibiotics.




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