•
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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