An Atypical Initial Presentation of Chronic Myeloid Leukemia with Central Nervous System and Lymph Node Blast Crises

Abstract

We describe the case of a young man with therapy-naive chronic myeloid leukemia who did not initially have any peripheral blood or bone marrow excess blasts but presented with extramedullary myeloid blast crises involving the central nervous system and multiple lymph nodes. Conventional cytogenetic tests were positive for t(9;22)(q34:q11) as well as for trisomy 8, 14 and 21 and del(16q). The patient’s peripheral blood and bone marrow were positive for the BCR-ABL oncogene when analyzed by fluorescence in situ hybridization and polymerase chain reaction. He achieved good clinical, radiological, cytogenetic and molecular response to acute myeloid leukemia induction chemotherapy combined with 16 doses of triple intrathecal chemotherapy and oral dasatinib (second-generation tyrosine kinase inhibitor) treatment. Due to his poor general condition, he was treated with 24 Gy of whole-brain radiation therapy, as allogeneic stem cell transplantation was not feasible. Although extramedullary CNS blast crises are usually associated with a very poor outcome, our patient remains in complete cytogenetic and molecular remission, on single-agent dasatinib, 4 years after the diagnosis with no current evidence of active extramedullary disease. This suggests that dasatinib has a role in controlling not only chronic-phase chronic myeloid leukemia, but also its CNS blast crisis.

Sensorineural Hearing Loss in Juvenile CML: A Rare Case Report in Surabaya, Indonesia

“… 14 CNS blast crisis is often associated with meningitis- or encephalitis-related clinical and radiological characteristics, including headache, ataxia, apahasia, seizures, and meningismus, and localized neurological symptoms and signs like. 13 , 21 Myeloid or lymphoid blasts are detected in the cerebrospinal fluid (CSF) and, in certain cases, molecular testing of the CSF reveals typical BCR-ABL oncogenes. 13 Unfortunately, in our patient, the presence of myeloblasts and the BCR-ABL oncogene in the CSF had not been established.…”

Prolonged Survival of a Patient with Chronic Myeloid Leukemia in Accelerated Phase with Recurrent Isolated Central Nervous System Blast Crisis

“…One patient diagnosed with chronic phase CML in October 2014 and treated with 100 mg/day dasatinib experienced 2 CNS relapses, the first in June 2015, which was successfully treated with 140 mg/day dasatinib plus intrathecal chemotherapy while BM was in chronic phase; and the second in September 2015, which was treated with 24 Gy radiation followed by unrelated 10/10 matched donor allo-SCT [ 24 ]. Another study described a patient with chronic phase CML who initially presented with extramedullary CNS and lymph node blast crises that were treated with dasatinib 70 mg orally twice daily and a standard AML induction regimen along with 16 doses of intrathecal methotrexate/cytarabine/hydrocortisone and 24 Gy of radiation [ 25 ]. Because of the patient’s poor general condition, allo-SCT was not advisable.…”

“…Because of the patient’s poor general condition, allo-SCT was not advisable. The patient remained in complete cytogenetic and molecular remission, on single-agent dasatinib for 4 years with no evidence of active extramedullary disease according to his most recent brain MRI performed 4 months prior to the writing of this report [ 25 ]. Splenomegaly persisted for some time in our patient, despite her being in CMR, and disappeared following treatment with PegINF-α2a, which acts as an immunotherapeutic agent.…”

Chronic Myeloid Leukemia Presenting with Fleeting Facial Nerve Palsy

“…Central nervous system (CNS) involvement in blast phase of CML presents with features like headache, vomiting, visual blurring, and cranial nerve palsy [1,2]. The commonest cranial nerve involved is seventh but third and sixth cranial nerve involvement has also been reported [3,4]. However, CML presenting with fleeting bilateral facial nerve palsy as the isolated presentation is very unusual.…”

“…Our patient was receiving corticosteroids and never underwent blood investigation and these may be responsible for late diagnosis. The diagnosis of CNS involvement may be suspected clinically or on radiological imaging studies but requires demonstration of blast cells in CSF for confirmation [1][2][3][4]. BCR-ABL transcript has also been demonstrated in CSF and when available may add to the diagnosis [5].…”