Myeloid Sarcoma as the Presenting Symptom of Chronic Myelogenous Leukemia Blast Crisis

Levy, Rebecca; Mardones, Mabel A.; Burch, Micah; Krause, John R.

doi:10.1080/08998280.2014.11929127

Cited by 16 publications

(18 citation statements)

References 18 publications

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“…Therefore, we recommend being aware of the increased thrombotic risk in severe forms and suggest an antithrombotic prophylaxis in cases with massive intravascular hemolysis and/or splenectomised, although prospective studies are advisable to give precise recommendations. The mortality rate in the severe cases presented was about 50%, greater than that of AIHA series reported in the literature (5-11%) [2,3]; although limited by the number of patients, this finding underlines that primary forms may be life-threatening, even more than that frequently observed in AIHA secondary to lymphoproliferative diseases [5] and bone marrow transplantation [6]. In our cases, death was due to sepsis and multiple organ failure, and was related with multitreatment, particularly splenectomy, and with concomitant thrombocytopenia.…”

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confidence: 60%

Effectiveness of lenalidomide in a patient with refractory anemia with ring sideroblasts and thrombocytosis with JAK2 (V617F) mutation

2015

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confidence: 60%

Effectiveness of lenalidomide in a patient with refractory anemia with ring sideroblasts and thrombocytosis with JAK2 (V617F) mutation

2015

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“…The acute extramedullary transformation of CML is defined as the infiltration of leukemia blasts into regions other than the bone marrow, which has been reported in only 4 to 16% of cases of CML [4].…”

Section: Discussionmentioning

confidence: 99%

Extensive Bone Lesions and Skin Lesions Revealing an Acute Transformation of an Extramedullary Chronic Myeloid Leukemia

Houssou¹,

Lamchahab²,

Hadri³

et al. 2018

CRJ

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Extramedullary blast transformation as the initial presentation of Chronic Myeloid Leukemia with bone marrow remaining in the chronic phase is an unusual event. We report the case of a 30-year-old with chronic CML at the medullary level and an acute transformation revealed by extensive bone lyses and skin lesions. A 30-year-old man had cutaneous nodules, functional impotence and paresis. Radiological investigations revealed osteolytic lesions of the right humeral head, right clavicle, ribs, C5 and C7 vertebrae with medullary compression. Anatomo-pathological study and immunohistochemistry of nodules and osteolytic lesions resulted in granulocytic sarcoma with CD10 +, CD34 +, CD13 +, CD33 +, CD117 and MPO positive. The myelogram was in favor of chronic phase CML. He was put on imatinib, the chemotherapy could not be started because his condition was impaired and he dies by metabolic disorder. The acute transformation of chronic phase CML into extra marrow remains unusual. The care is a real challenge hence the need for consensual recommendations.

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“…Extramedullary blast crisis of CML is defined by infiltration of leukemic blasts in areas other than bone marrow, which has been reported in only 4–16% of CML cases during the disease course [4]. As shown in Table 1, CML cases presenting with a bilineal extramedullary disease with bone marrow in chronic phase are rare, with only 4 reported cases.…”

Section: Discussionmentioning

confidence: 99%

Bilineal Extramedullary Blast Crisis as an Initial Presentation of Chronic Myeloid Leukemia: A Case Report and Literature Review

Gao

Wang

et al. 2016

Am J Case Rep

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Patient: Male, 49Final Diagnosis: T-lymphoid/myeloid bilineal blastic transformation of CMLSymptoms: Rapidly enlarging mass in left neckMedication: —Clinical Procedure: Biopsy of the left submandibular lymph nodesSpecialty: HematologyObjective:Rare co-existance of disease or pathologyBackground:Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder characterized by the Philadelphia chromosome generated by the reciprocal translocation t(9: 22)(q34;q11). CML is usually diagnosed in the chronic phase. Blast crisis represents an advanced phase of CML. Extramedullary blast crisis as the initial presentation of CML with bone marrow remaining in chronic phase is an unusual event. Further, extramedullary blast crisis with T lymphoid/myeloid bilineal phenotype as an initial presentation for CML is extremely unusual.Case Report:Here, we report the case of a 49-year-old male with rapidly enlarged submandibular lymph nodes. Biopsy specimen from the nodes revealed a characteristic appearance with morphologically and immunohistochemically distinct myeloblasts and T lymphoblasts co-localized in 2 adjacent regions, accompanied by chronic phase of the disease in bone marrow. The presence of the BCR/ABL1 fusion gene within both cellular populations in this case confirmed the extramedullary disease represented a localized T lymphoid/myeloid bilineal blastic transformation of CML. After 3 courses of combined chemotherapy plus tyrosine kinase inhibitor treatment, the mass was completely regressed with a 3-log decrease in BCR/ABL1 transcript from baseline. Five months after the diagnosis, the patient showed diminished vision, hand tremors, and weakness of lower extremities. Flow cytometric immunophenotyping of cerebrospinal fluid revealed the presence of myeloid blasts. An isolated central nervous system relapse of leukemia was identified. Following high-dose systemic and intrathecal chemotherapy, the patient continued to do well.Conclusions:The possibility of extramedullary blast crisis as an initial presentation in patients with CML should be considered. Further, an isolated central nervous system blast crisis should be considered if neurological symptoms evolve in patients who have shown a good response to therapy.

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Myeloid Sarcoma as the Presenting Symptom of Chronic Myelogenous Leukemia Blast Crisis

Cited by 16 publications

References 18 publications

Effectiveness of lenalidomide in a patient with refractory anemia with ring sideroblasts and thrombocytosis with JAK2 (V617F) mutation

Effectiveness of lenalidomide in a patient with refractory anemia with ring sideroblasts and thrombocytosis with JAK2 (V617F) mutation

Extensive Bone Lesions and Skin Lesions Revealing an Acute Transformation of an Extramedullary Chronic Myeloid Leukemia

Bilineal Extramedullary Blast Crisis as an Initial Presentation of Chronic Myeloid Leukemia: A Case Report and Literature Review

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