Agnogenic myeloid metaplasia with pleural extramedullary leukemic transformation

Hassan F. Nadrous, Michael J. Krowka, Rebecca F. McClure, Ayalew Tefferi, Kaiser G. Lim

Research output: Contribution to journalArticlepeer-review

11 Scopus citations


Agnogenic myeloid metaplasia (AMM) is one of the myeloproliferative disorders, and is usually accompanied by extramedullary hematopoiesis (EMH) in various organs, mainly in the liver, spleen and lymph nodes. Extramedullary hematopoiesis and/or leukemic transformation of EMH in the pleura is a rare occurrence and is usually asymptomatic. Pleural involvement is usually diagnosed on postmortem examination. Herein we describe a 71-year-old man with newly diagnosed agnogenic myeloid metaplasia who was evaluated for progressively worsening dyspnea, pulmonary hypertension and bilateral pleural effusions. EMH involving the lungs and pleura was suspected. A sulfur colloid technetium 99m bone marrow scan performed to detect extramedullary hematopoiesis was negative. The diagnostic thoracentesis yielded bloody fluid that contained a large population of myeloblasts, indicating pleural leukemic transformation. The patient received 100 cGy to the whole lung for treatment of pulmonary hypertension due to EMH. This was followed by 1500 cGy total dose of radiation to the left lung for pleural extramedullary leukemic transformation. Pleural effusions resolved and repeat echocardiography showed reduction of the pulmonary artery pressure. Three months later he had leukemic transformation involving the skin and lymph nodes. Four months after radiation therapy, he had full-blown acute myeloid leukemia. He received 2 cycles of Gemtuzumab ozogamicin (MylotargR), allopurinol and hydroxyurea. Three months after initiation of chemotherapy, he deteriorated and received salvage chemotherapy of prednisone, VP-16 and imatinib mesylate (GleevecR). He was hospitalized for neutropenic fever and was diagnosed to have pulmonary aspergillosis. He died of multisystem failure 8 1/2 months after being diagnosed with AMM.

Original languageEnglish (US)
Pages (from-to)815-818
Number of pages4
JournalLeukemia and Lymphoma
Issue number4
StatePublished - Apr 1 2004


  • Agnogenic myeloid metaplasia
  • Extramedullary hematopoiesis
  • Leukemia
  • Pulmonary hypertension

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research


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