Abstract
Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by immature granulocytes in peripheral blood and bone marrow. In 95% of cases, it is always due to the presence of Philadelphia chromosome characterized by the presence of reciprocal translocation between chromosome 9 and 22. However, in 7% -17% of individuals, extramedullary proliferation also occurs, either in skin, lymph nodes, bone or central nervous system (CNS), which could be either myeloid, lymphoid or mixed progenitor in origin. The present case is of a 23-year-old male who presented with lower limb weakness, bowel and urinary incontinence. His complete blood count (CBC) findings showed a raised white blood count (WBC) of 408 X 10E9/L. Peripheral film, bone marrow biopsy and immunohistochemistry showed findings consistent with CML in chronic phase. Bone marrow cytogenetic revealed the presence of Philadelphia chromosome. Simultaneously, magnetic resonance imaging (MRI) was done which revealed extradural mass at L1-L3 level; histopathological and immunohistochemistry findings showed features compatible with precursor B cell lymphoblastic lymphoma. His cerebrospinal fluid (CSF) cytology revealed similar blast cells. This extramedullary presence of lymphoid blast cells in the CNS put the patient in the rare entity of CML in blast crisis. He was started on tablet nilotinib and also received multiple cycles of intrathecal chemotherapy with cytosar, methotrexate and hydrocortisone. He also underwent radiotherapy of extradural mass. His lower limb weakness improved dramatically. However, after receiving the fourth cycle of intrathecal therapy, the patient died consequent to neutropenic sepsis. Extramedullary blast crisis in CML has a poor prognosis. Any patient with CML, presenting with CNS symptoms or lymph node enlargement should be thoroughly investigated for extramedullary blast crisis, as there is a considerable change in management and prognosis from the prototype CML in chronic phase.
Highlights
Extramedullary presentation of chronic myeloid leukemia (CML) in the form of myeloid sarcoma has been reported in several literature [1,2]
Magnetic resonance imaging (MRI) was done which revealed extradural mass at L1-L3 level; histopathological and immunohistochemistry findings showed features compatible with precursor B cell lymphoblastic lymphoma. His cerebrospinal fluid (CSF) cytology revealed similar blast cells. This extramedullary presence of lymphoid blast cells in the central nervous system (CNS) put the patient in the rare entity of CML in blast crisis
Any patient with CML, presenting with CNS symptoms or lymph node enlargement should be thoroughly investigated for extramedullary blast crisis, as there is a considerable change in management and prognosis from the prototype CML in chronic phase
Summary
Extramedullary presentation of chronic myeloid leukemia (CML) in the form of myeloid sarcoma has been reported in several literature [1,2]. Concomitant presentation of CML in bone marrow along with the simultaneous presence of precursor B cell lymphoblastic lymphoma in extramedullary tissue site has rarely been cited. How to cite this article Mansoori H, Faraz M, Qadir H, et al (October 17, 2020) Precursor Lymphoblastic Lymphoma in the Extramedullary Tissue: A Rare Manifestation of Chronic Myeloid Leukemia in Blast Crisis. MRI lumbosacral spine revealed extradural mass at L1-L3 level; laminectomy was done Histopathological findings of this extradural mass showed sheets of small-sized neoplastic cells with scant cytoplasm, round nuclei with coarse chromatin (Figure 2, hematoxylin and eosin; X200 ). Cytology revealed a large number of atypical cells with mildly enlarged nuclei, irregular nuclear contour and scanty cytoplasm This patient was diagnosed with CML with extramedullary blast crisis, presenting as CNS leukemia. The patient was readmitted on third day after receiving fourth dose of triple Intrathecal therapy, with febrile neutropenia (WBC:0.2) and died after two days due to E-coli sepsis
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