weekly_question 27/8/2023

[Admin]

A three-year old male with hereditary spherocytosis is referred for a discussion regarding partial splenectomy. Which of the following are appropriate expecations following partial splenectomy?

A a 20% chance of needing completion splenectomy

B an increase in baseline hemoglobin

C decreased transfusion requirements

D an increase in reticulocyte count

[Admin]

Correct answer
C decreased transfusion requirements

Hereditary spherocytosis (HS) is a relatively common inherited hemoglobinopathy. Its clinical manifestations are usually minor and only a small subset require transfusions or other complications of anemia. Few patients have severe anemia and benefit from splenectomy.

Splenectomy dramatically decreases the frequency of sequestration events and transfusions from hypersplenism in patients with severe HS. Concerns regarding splenectomy include the loss of immunoglobulin function and the subsequent increased risk of overwhelming post splenectomy sepsis. In addition, the ongoing hemolysis seems to predispose the postsplenectomy patient to long term risks of pulmonary hypertension.

Partial splenectomy leaves the short gastric vessels attached to the superior pole of the spleen while removing 90% of the gland after dividing the hilar vessels. This procedure has been shown in small series at multiple institutions to decrease reticulocyte counts and sequestration events, increase the Hgb level and preserve immune function as evidenced by persistent IgM and IgG levels postsplenectomy.

Complete or partial splenectomy is not indicated in children with mild HS since transfusions and other complications are rare. Completion splenectomy is not common in the intermediate period (up to six year follow-up); however, its necessity in the long term is unknown.

[Ismailmohamed]

B

[Osama elshafie]

B

[Ahmed Rabee]

B

[Admin]

Correct answer
C decreased transfusion requirements

Hereditary spherocytosis (HS) is a relatively common inherited hemoglobinopathy. Its clinical manifestations are usually minor and only a small subset require transfusions or other complications of anemia. Few patients have severe anemia and benefit from splenectomy.

Splenectomy dramatically decreases the frequency of sequestration events and transfusions from hypersplenism in patients with severe HS. Concerns regarding splenectomy include the loss of immunoglobulin function and the subsequent increased risk of overwhelming post splenectomy sepsis. In addition, the ongoing hemolysis seems to predispose the postsplenectomy patient to long term risks of pulmonary hypertension.

Partial splenectomy leaves the short gastric vessels attached to the superior pole of the spleen while removing 90% of the gland after dividing the hilar vessels. This procedure has been shown in small series at multiple institutions to decrease reticulocyte counts and sequestration events, increase the Hgb level and preserve immune function as evidenced by persistent IgM and IgG levels postsplenectomy.

Complete or partial splenectomy is not indicated in children with mild HS since transfusions and other complications are rare. Completion splenectomy is not common in the intermediate period (up to six year follow-up); however, its necessity in the long term is unknown.