Patient Presentation
A 35-year-old woman presents with severe fatigue, jaundice, and an enlarged spleen on physical examination. She carries a known diagnosis of hereditary spherocytosis and has suffered frequent hemolytic episodes with recurrent anemia.
Laboratory studies show a low hemoglobin and elevated indirect bilirubin consistent with ongoing hemolysis. She has required multiple transfusions over the years.
You are asked to evaluate her for possible splenectomy and to counsel her on the indications, preoperative preparation, operative approach, and long-term risks of asplenia.
What You'll Be Asked — and What a Strong Resident Discusses
-
What is the pathophysiology of hereditary spherocytosis and why does the spleen drive the hemolysis?
Expected answer
HS is a congenital RBC membrane cytoskeletal defect (spectrin/ankyrin) that produces rigid, non-deformable spherocytes. These abnormal cells cannot navigate the splenic cords and sinusoids, so they are sequestered and destroyed by the spleen, causing chronic extravascular hemolysis, anemia, jaundice from unconjugated bilirubin, splenomegaly, and a predisposition to pigment gallstones. Splenectomy removes the primary site of red cell destruction while the intrinsic membrane defect persists.
-
What are the accepted indications for splenectomy in hereditary spherocytosis?
Expected answer
Splenectomy is indicated for recurrent transfusion requirements and for intractable/symptomatic hemolysis (including intractable leg ulcers and severe symptomatic anemia). This patient with frequent hemolytic episodes and repeated transfusions meets criteria. Splenectomy dramatically reduces hemolysis and transfusion needs even though the membrane defect remains.
-
How does the indication for splenectomy differ across other benign RBC disorders?
Expected answer
In pyruvate kinase deficiency, recurrent transfusion requirement is the indication. In warm-antibody autoimmune hemolytic anemia, splenectomy is offered after failure of medical therapy (steroids). Importantly, splenectomy is NOT indicated in G6PD deficiency — management is avoidance of oxidative triggers. Recognizing that not every hemolytic anemia benefits from splenectomy is key.
-
What preoperative preparation is essential before an elective splenectomy?
Expected answer
Vaccinate against the encapsulated organisms — Streptococcus pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis — ideally at least 2 weeks before elective surgery. Optimize hemoglobin (transfuse if needed), and evaluate for gallstones since HS patients frequently have pigment stones; concomitant cholecystectomy is considered if stones are present. Counsel on lifelong risk of overwhelming post-splenectomy infection (OPSI).
-
What is the preferred operative approach and key steps?
Expected answer
Laparoscopic splenectomy is the preferred approach for most elective, non-massive spleens because of reduced morbidity and faster recovery. Key steps: secure vascular control at the hilum (splenic artery and vein), take the short gastric vessels along the greater curve carefully to avoid gastric injury, mobilize the splenic ligaments (splenophrenic, splenorenal, splenocolic), and search deliberately for accessory spleens. Open splenectomy is chosen for massive splenomegaly or when laparoscopy is unsafe.
-
Why is a search for accessory spleens important, and where are they found?
Expected answer
Accessory splenic tissue occurs in roughly 15–30% of patients and, if left behind, can hypertrophy and cause recurrent hemolysis or relapse of ITP. The most common location is the splenic hilum, followed by the splenic pedicle, gastrosplenic and splenocolic ligaments, greater omentum, and along the tail of the pancreas. A systematic exploration is mandatory.
-
Contrast the surgical role in ITP versus TTP.
Expected answer
ITP is the most common indication for elective splenectomy: when platelets are <30,000, medical therapy (prednisone for 2–4 weeks with taper, ± IVIG) is first-line, and splenectomy is reserved for failure of medical therapy or recurrent disease. In TTP, plasmapheresis is first-line therapy; splenectomy is reserved for patients with excessive/refractory plasma exchange requirements. Both are elective decisions predicated on failure of medical management.
-
How do you counsel the patient about long-term risks after splenectomy?
Expected answer
The main concern is overwhelming post-splenectomy infection (OPSI) from encapsulated organisms, which is low-frequency but can be rapidly fatal. Lifelong precautions include up-to-date vaccinations with boosters, prompt medical attention and early empiric antibiotics for febrile illness, consideration of standby antibiotics, and malaria/animal-bite precautions. There is also an increased risk of postoperative venous/portal thrombosis, especially with thrombocytosis.
-
What indications for splenectomy exist in malignant and 'other' conditions?
Expected answer
For WBC disorders (e.g., non-Hodgkin lymphoma) splenectomy is indicated for symptomatic splenomegaly and cytopenias; bone marrow disorders (CML, myelofibrosis, polycythemia vera) generally warrant splenectomy for symptomatic splenomegaly. Among 'other' lesions, splenectomy is the treatment of choice for splenic abscess and symptomatic parasitic cysts, whereas symptomatic nonparasitic cysts can be managed with partial splenectomy or unroofing.
What Residents Often Miss
- Recommending splenectomy for G6PD deficiency, where it is not indicated — management is avoidance of oxidative triggers.
- Failing to vaccinate against encapsulated organisms (pneumococcus, H. influenzae type b, meningococcus) at least 2 weeks before elective splenectomy.
- Neglecting to search for and remove accessory spleens, leading to recurrent hemolysis or ITP relapse.
- Not evaluating for or addressing pigment gallstones in a chronic hemolysis patient, missing an opportunity for concurrent cholecystectomy.
- Proceeding to splenectomy in ITP or TTP without first documenting failure of appropriate medical therapy (steroids for ITP, plasmapheresis for TTP).
- Forgetting to counsel on lifelong OPSI risk and the plan for early antibiotics with any febrile illness.
- Overlooking postoperative thrombocytosis and the increased risk of portal/splenic vein thrombosis requiring VTE prophylaxis.
Want More Practice?
Run this case live with an AI examiner that pushes back, asks for operative detail, and grades you on the ABS competencies — then gives instant feedback.
Start practicing freeNo credit card required · 5 free practice cases