Development of myelofibrosis and acute monocytic leukemia in a patient with hereditary spherocytosis: A case report

Medicine (Baltimore). 2019 Dec;98(50):e18266. doi: 10.1097/MD.0000000000018266.


Introduction: Common symptoms of hereditary spherocytosis (HS) include intermittent jaundice and splenomegaly. Here, we present an unusual clinical course wherein a patient with HS treated with splenectomy developed secondary myelofibrosis and acute monocytic leukemia (M5).

Patient concerns: After presenting with paleness, fatigue and jaundice, the patient was diagnosed with HS. After splenectomy, follow-up testing, including bone marrow biopsy, revealed myelofibrosis. Subsequently, the patient exhibited blood cell abnormalities consistent with M5.

Diagnosis: M5 comorbid with myelofibrosis and a history of HS.

Interventions: HS was treated with splenectomy. Myelofibrosis was treated with hydroxyurea. The patient refused chemotherapy for M5 and was discharged. He was maintained on hydroxyurea and received periodic blood product transfusions with regular routine blood test monitoring.

Outcomes: Because of intracranial hemorrhage, the patient died on May 17, 2018, a little >10 months after being diagnosed with leukemia.

Conclusion: The present patient developed M5 while undergoing treatment for myelofibrosis and after undergoing splenectomy for HS, raising the question of whether these conditions might be associated. Examination of this question will require the analysis of additional cases.

Publication types

  • Case Reports

MeSH terms

  • Biopsy
  • Follow-Up Studies
  • Humans
  • Hydroxyurea / therapeutic use
  • Leukemia, Monocytic, Acute / complications*
  • Leukemia, Monocytic, Acute / diagnosis
  • Male
  • Middle Aged
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / drug therapy
  • Primary Myelofibrosis / etiology*
  • Spherocytosis, Hereditary / complications*
  • Spherocytosis, Hereditary / diagnosis
  • Spherocytosis, Hereditary / surgery
  • Splenectomy / adverse effects*
  • Time Factors
  • Treatment Outcome


  • Hydroxyurea