Post-Operative Care for Myelofibrosis Splenectomy
Immediate postoperative management must focus on preventing the three major life-threatening complications: extreme thrombocytosis with thrombosis (occurring in ~22% of patients), overwhelming post-splenectomy infection (OPSI), and hemorrhage, which collectively account for the 50% complication rate and 5-10% mortality in this population. 1
Immediate Postoperative Period (Days 0-7)
Thrombotic Prophylaxis and Platelet Management
Initiate therapeutic anticoagulation with low molecular weight heparin (LMWH) immediately postoperatively and continue for extended duration given the extremely high thrombotic risk in myelofibrosis patients 2, 3
Monitor platelet counts daily for the first week, as extreme thrombocytosis (>1000 × 10⁹/L) develops rapidly and is associated with increased perioperative thrombosis and decreased survival 4, 5
If platelet count rises above 400 × 10⁹/L, immediately initiate cytoreductive therapy with hydroxyurea to prevent thrombotic complications 1, 2
Consider platelet apheresis for extreme thrombocytosis (>1000 × 10⁹/L) combined with prompt cytoreduction, which has been shown to decrease thrombohemorrhagic complications 4
Maintain mechanical prophylaxis with sequential compression devices throughout hospitalization 2
Infection Prevention and Surveillance
Administer prophylactic antibiotics (phenoxymethylpenicillin 250-500 mg twice daily or erythromycin 500 mg twice daily if penicillin-allergic) starting immediately postoperatively and continue lifelong 1, 2
Monitor closely for fever, as any temperature >38°C (101°F) represents a medical emergency requiring immediate broad-spectrum antibiotics with third-generation cephalosporins before culture results 1, 3
Recognize that overwhelming post-splenectomy infection (OPSI) from encapsulated organisms (Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae) carries mortality rates approaching 50% 2, 3
Hemorrhage Monitoring
Monitor surgical site and drain output closely for bleeding, as hemorrhagic complications occur in 14% of patients and account for 6.7% of fatal complications 4
Recognize the paradox that these patients face both thrombotic and hemorrhagic risks simultaneously due to the underlying myeloproliferative disorder 1
Intermediate Postoperative Period (Weeks 1-4)
Monitoring for Specific Myelofibrosis Complications
Assess for accelerated hepatomegaly, which occurs in 16% of post-splenectomy myelofibrosis patients as extramedullary hematopoiesis shifts to the liver 1, 5
Monitor for subphrenic abscess formation through clinical examination and imaging if fever or abdominal pain develops 1
Evaluate peripheral blood for leukocytosis with excess blasts, as blast transformation risk increases post-splenectomy, particularly in patients with preoperative thrombocytopenia 1, 5, 6
Hematologic Response Assessment
Assess transfusion requirements at 1 month and 6 months post-splenectomy, recognizing that only 23% of patients achieve durable remission from transfusion-dependent anemia 5, 6
Monitor for improvement in constitutional symptoms, which respond in 67% of patients 5
Understand that responses in cytopenias are often transient, with durable responses at 6 months occurring in only a minority of patients 6
Long-Term Management (Beyond 1 Month)
Lifelong Infection Prevention
Ensure patient carries medical alert identification (card, bracelet, or pendant) indicating asplenic status 1
Provide home supply of antibiotics (penicillin VK, erythromycin, or levofloxacin) for immediate self-administration if fever develops 1
Educate patient that any fever >38°C (101°F) requires immediate emergency department evaluation, as OPSI can progress to septic shock within hours 1, 2
Administer reimmunization every 5-10 years with pneumococcal, meningococcal, and H. influenzae vaccines 2
Disease Progression Monitoring
Monitor for blast transformation, which occurs in 16% of post-splenectomy patients and is more common with preoperative thrombocytopenia 5, 6
Recognize that median post-splenectomy survival is only 10-27 months, reflecting the advanced nature of disease requiring surgical intervention 5, 6
Continue cytoreductive therapy as needed to maintain platelet count <400 × 10⁹/L 1
Portal and Caval Thrombosis Management
If portal or caval venous thrombosis develops, initiate prolonged warfarin anticoagulation for 3-6 months 3
Recognize that portal vein thrombosis occurs more commonly in myeloproliferative disease patients post-splenectomy 3
Critical Pitfalls to Avoid
Never discontinue thrombotic prophylaxis prematurely, as thrombotic complications occur in approximately 10% of patients and can be fatal 1, 4
Never delay cytoreduction for rising platelet counts, as extreme thrombocytosis is directly associated with decreased survival 4, 5
Never assume fever is benign in an asplenic patient—this represents OPSI until proven otherwise and requires immediate empiric antibiotics 1, 3
Do not expect splenectomy to prolong survival—the procedure is purely palliative, with median survival of 10-27 months post-operatively 7, 5, 6
Recognize that preoperative thrombocytopenia (<100 × 10⁹/L) is a marker of advanced disease with increased blast transformation risk and inferior survival, though this does not negate the palliative benefits of surgery 4, 5