Splenectomy is most successful in
|B||Chronic myeloid leukemia|
|C||Idiopathic thrombocytopenic purpura|
a. Hereditary spherocytosis is an autosomal dominant disease that results from a deficiency of spectrin, a red blood cell cytoskeletal protein.
b. This protein defect causes a membrane abnormality in the red blood cells resulting in small, spherical, and rigid erythrocytes. These cells have increased osmotic fragility.
c. These spherocytes are more susceptible to becoming trapped in the spleen and destroyed.
d. The clinical features of this disease include anemia, occasionally with jaundice, and splenomegaly.
e. The diagnosis is made by identification of spherocytes on the peripheral blood smear, an increased reticulocyte count, increased osmotic fragility, and a negative Coombs test.
f. Splenectomy decreases the rate of hemolysis and usually leads to resolution of the anemia.
g. Although splenectomy does not normalize the morphology of the red blood cells, it does reduce the trapping and premature destruction of them.
h. It is generally recommended that the operation be delayed until after the fourth year of life to preserve immunologic function of the spleen in young children who are most at risk for OPSI.
i. If gallstones are present, it is appropriate to perform cholecystectomy at the time of the splenectomy
j. Other anemias associated with erythrocyte structural abnormalities include hereditary elliptocytosis, hereditary pyropoikilocytosis, hereditary xerocytosis, and hereditary hydrocytosis.
k. Splenectomy is indicated for the severe hemolytic anemia that commonly occurs in these conditions.
Overwhelming Postsplenectomy Infection (OPSI)
• OPSI is the MC fatal late complication of splenectomy.
- Infection may occur at any time after splenectomy
- In one recent series, most infections occurred more than 2 years after splenectomy and 42% occurred more than 5 years after splenectomy.
• OPSI typically begins with a prodromal phase characterized by fever, rigors and chills and other nonspecific symptoms, including sore throat, malaise, myalgias, diarrhea, and vomiting.
• Many patients have no identifiable focal site of infection and present only with high-grade primary bacteremia.
- Progression of the illness is rapid, with the development of hypotension, disseminated intravascular coagulation, respiratory distress, coma, and death within hours of presentation,
- Despite antibiotics and intensive care, the mortality rate is between 50-70% for florid OPSI.
Complications of Splenectomy
• Left lower lobe atelectasis: MC complication
• Pleural effusion
• Portal vein thrombosis
- DVT prophylaxis is routinely recommended".
- In patients with hemolytic anemia or myeloproliferative disorders and splenomegaly, thrombotic risk is heightened, particularly the risk of portal vein thrombotic.
- Patients undergoing splenectomy for malignancy or myeloproliferative disorders should be strongly considered for peri operative pharmaco prophylaxis, either LMWH or unfractionated heparin.
• Subphrenic hematoma
• Subphrenic abscess
• Wound infection
• Pancreatic fistula