Hyposplenism its causes,diagnosis,complication, prevention and its prognosis

Hyposplenism

•         


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.

Howell Jolly bodies

•          Persistant 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

•          Sickle cell anemia

•          Gluten induced enteropathy (Coeliac disease).

•          Crohin's Disease and Ulcerative colitis

•          Myelofibrosis and essential thrombocytosis

•          SLE and RA

•          Lymphoma and Multiple Myeloma

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