Chronic myelogenous leukemia initially presenting with multiple subcutaneous tumors due to extramedullary hematopoiesis

• Published in: Rinshō ketsueki Vol. 57; no. 4; p. 461
• Main Authors: Kurosawa, Shuhei, Doki, Noriko, Kaito, Satoshi, Sakaguchi, Masahiro, Harada, Kaito, Hino, Yutaro, Yamamoto, Keita, Ikegawa, Shuntaro, Yosioka, Kosuke, Watanabe, Daisuke, Senoo, Yasushi, Watakabe, Kyoko, Hagino, Takeshi, Igarashi, Aiko, Najima, Yuho, Kobayashi, Takeshi, Kakihana, Kazuhiko, Miyawaki, Shuichi, Sakamaki, Hisashi, Ohashi, Kazuteru
• Format: Journal Article
• Language: Japanese
• Published: Japan 01.04.2016
• Subjects: Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Female; Hematopoiesis, Extramedullary; Humans; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - drug therapy; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - genetics; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - pathology; Middle Aged; Protein-Tyrosine Kinases - antagonists & inhibitors; Remission Induction; Skin Neoplasms - drug therapy; Skin Neoplasms - genetics; Skin Neoplasms - pathology
• ISSN: 0485-1439
• DOI: 10.11406/rinketsu.57.461

A 53-year-old woman was admitted with right upper-extremity pain and multiple subcutaneous masses. Bone marrow aspirate showed hypercellular marrow with increased myeloid components at all stages of maturation. Cytogenetic analysis of the bone marrow revealed 100% Philadelphia chromosome positivity along with BCR/ABL gene rearrangement, as demonstrated by polymerase chain reaction (PCR). A diagnosis of chronic phase of chronic myeloid leukemia (CML) was therefore made. Biopsy of one of the subcutaneous masses showed proliferation of granulocytes in various stages of differentiation. There were also erythroid cells and megakaryocytes, without p53 and CD34-positive blasts. These results suggested that the subcutaneous masses had developed from extramedullary hematopoiesis, not blastomas. The patient was administered dasatinib (DA) 140 mg, combined with radiation therapy for pain and peripheral neuropathy from the right axial extramedullary tumor. The patient showed complete hematological remission and the subcutaneous masses had disappeared 1 month after starting administration of DA. Because the patient did not achieve a cytogenetic response, the tyrosine kinase inhibitor nilotinib was administered. She will undergo allogeneic stem cell transplantation in the near future. Extramedullary hematopoiesis in the early stages of CML is uncommon. Our case emphasizes the need to elucidate the pathogenesis of extramedullary hematopoiesis in the early stages of CML.