Biphenotypic Extramedullary Blast Crisis as a Presenting Manifestation of Philadelphia Chromosome-Positive CML in a Child

Ganessan, K; Goel, Ruchika; Kumar, Kavitha Ashok; Bakhshi, Sameer

doi:10.1080/08880010701198787

Cited by 13 publications

(13 citation statements)

References 11 publications

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“…Literature review shows few rare case reports too in which immunohistochemistry of extra medullary granulocytic sarcoma had shown T cell lineage extra medullary blast crisis [2,[10][11][12][13]. Extra medullary blast crisis with biphenotypic picture (mixed myeloid and T cell features) were also reported separately by Hossain et al [14],Cervantes et al [15], Ganessan et al [16] and Chen et al [17].…”

Section: Discussionmentioning

confidence: 99%

Chronic Myeloid Leukemia with Extramedullary Blast Crisis: Two Unusual Sites with Review of Literature

Sahu

Malhotra

Uthamalingam

et al. 2014

Indian J Hematol Blood Transfus

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Extramedullary blast crisis (EBC) in chronic myeloid leukemia (CML) is a rare phenomenon and represents infiltration of leukemic blasts in areas other than bone marrow. Lymph node is the most common site of involvement by EBC. We herein present a case of CML who suffered from two discrete episodes of EBC at atypical locations (scalp and paravertebral) within an interval duration of nine months. A-38-year-old female was diagnosed as a case of CML with extramedullary blast crisis in scalp at presentation. She received treatment with imatinib 600 mg once daily through Novartis Oncology Access Program (NOA). She achieved hematological remission. However nine months later she was readmitted with spinal shock due to cord compression secondary to paraspinal chloroma. She was started on tablet Nilotinib in view of failure to 1st line therapy. Her compressive myelopathy was treated with pulses of high dose dexamethasone. However soon she died due to pneumonia. Case ReportA-38-year old lady was seen in February 2013 with complaints of scalp swelling in left parietal area of 5 9 5 cm in dimension (Fig. 1A). Rest of general physical examination was normal. Per abdomen examination revealed splenomegaly of 6 cm below left costal margin. Her total blood count showed hemoglobin-114 g/L, platelet count-417 9 10 9 /L, and total leukocyte count-146 9 10 9 /L with predominance of myeloid precursors -12 % myelocytes, 11 % metamyelocytes. Excisional biopsy of scalp swelling confirmed MPO positive chloroma (Fig. 2). Bone marrow examination showed hypercellular marrow spaces with 2 % blasts, 5 % basophils, 15 % myelocytes and 14 % metamyelocytes with mild megakaryocytic hyperplasia. BCR-ABL quantitative transcript ratio from blood was 78.35 %. Hence diagnosis of CML with extra medullary myeloid blast crisis of scalp was made. She was started on imatinib 600 mg OD. Second generation tyrosine kinase inhibitors (TKIs) or allogeneic stem cell transplantation were offered to her but she could not afford due to financial constraints. Reassessment at 3 months showed complete hematological remission with hemoglobin-113 gm/L, platelet count-135 9 10 9 /L, TLC-4.97 9 10 9 /L and no immature cells on differential count analysis. There was significant reduction in size of chloroma. Her quantitative transcript for BCR-ABL translocation was found to be 7.37 % at end of 6 months. The dose of imatinib was increased to 800 mg/day as BCR ABL transcripts levels were above 1 % at 6 months. However, 3 months later, she presented with complaints of sudden onset quadriplegia with bowel and bladder incontinence. Neurological examination revealed features suggestive of compressive myelopathy with power of 0/5 in bilateral lower limbs and pansensory loss below D4 level. An urgent CEMRI of dorsolumbar spine showed heterogeneously enhancing posterior epidural soft tissue component compressing and displacing the spinal cord at D4, 5, 6 and 7 level (Fig. 1B, C). In view of significant past history of scalp chloroma, clinical

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Section: Discussionmentioning

confidence: 99%

Chronic Myeloid Leukemia with Extramedullary Blast Crisis: Two Unusual Sites with Review of Literature

Sahu

Malhotra

Uthamalingam

et al. 2014

Indian J Hematol Blood Transfus

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“…5 An extramedullary blast crisis at presentation is even rarer with this phenomenon being cited in individual case reports. 6 Various signs and symptoms may occur as a result of the specific effect of an extramedullary blastic tumor. In this current case, the tumor involved the leptomeninges and the patient developed neurologic symptoms.…”

Section: Discussionmentioning

confidence: 99%

Extramedullary B Lymphoblastic Crisis of CML, Presenting as a Leptomeningeal Tumor - A Case Report -

et al. 2009

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We report here on a rare case of a patient who presented with an extramedullary B lymphoblastic crisis as an initial manifestation of chronic myelogenous leukemia (CML). A 71-year-old man visited the emergency room due to suddenly developed dysarthria and right side weakness. Emergency craniotomy was done under the presumptive diagnosis of subdural hemorrhage. During the operation, a poorly demarcated firm mass was identified in the leptomeningeal space. Microscopically, the majority of the tumor was composed of monotonous immature cells with blast morphology, and these cells were immunoreactive for TdT, CD34, CD10 and CD20, indicating the precursor B-cell phenotype. The peripheral area of the tumor consisted of myeloid cells in various stages of maturation, and these cells were reactive for myeloperoxidase, chloroacetate esterase, CD43 and CD15. FISH analysis using the LSI bcr-abl dual color probe showed gene fusion signals in both the B-lymphoblasts and myeloid cells. The peripheral blood and bone marrow findings were consistent with CML with no evidence of a blast crisis. Cytogenetic study of the bone marrow demonstrated the 46, XY, t(9;22)(q34;q11) chromosome. A diagnosis of extramedullary B lymphoblastic blast crisis in a patient with Philadelphia chromosome-positive CML was made. Despite treatment, the patient died 3 months after he was diagnosed.

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“…It took a rather long time for most cases to develop extramedullary blast crisis after their initial diagnoses [4,10,11,12,13,14]. Only 2 cases were diagnosed with blast crisis at the initial presentation [15,16]. In addition, Kim [17] described 2 cases of sudden extramedullary T lymphoblastic blast crisis, and it was suggested that the cessation of imatinib may have contributed to this phenomenon.…”

Section: Discussionmentioning

confidence: 99%

Sudden Extramedullary Blast Crisis of Chronic Myeloid Leukemia Manifesting as T-Cell Lymphoblastic Lymphoma

Wei¹,

Huang²,

Wang³

et al. 2013

Oncol Res Treat

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Background: Chronic myeloid leukemia (CML) is usually diagnosed in the chronic phase. An extramedullary blast crisis mimicking a T-cell lymphoma is a rare finding. Case Report: A 35-year-old man presented with multiple lymphadenopathy 2 months after diagnosis of Philadelphia chromosome (Ph)-positive CML in the chronic phase. Cervical lymph node biopsy later indicated an extramedullary blast crisis resembling T-cell lymphoblastic lymphoma (TLBL). The tumor mass was composed of primitive lymphoid cells expressing terminal deoxynucleotidyl transferase (TdT), CD3, CD43, CD5, CD99, and Bcl-2. Although the pathological diagnosis, confirmed by 2 independent pathological centers, was more typical of TLBL, fluorescence in situ hybridization (FISH) analysis showed the bcr-abl fusion gene within the blastic tumor cells. The FISH finding confirmed that the mass represented an extramedullary, immature blastic transformation of CML rather than a de novo T-cell lymphoma. Conclusion: The diagnosis of de novo TLBL should be suspected before excluding the extramedullary blast phase of CML.

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Biphenotypic Extramedullary Blast Crisis as a Presenting Manifestation of Philadelphia Chromosome-Positive CML in a Child

Cited by 13 publications

References 11 publications

Chronic Myeloid Leukemia with Extramedullary Blast Crisis: Two Unusual Sites with Review of Literature

Chronic Myeloid Leukemia with Extramedullary Blast Crisis: Two Unusual Sites with Review of Literature

Extramedullary B Lymphoblastic Crisis of CML, Presenting as a Leptomeningeal Tumor - A Case Report -

Sudden Extramedullary Blast Crisis of Chronic Myeloid Leukemia Manifesting as T-Cell Lymphoblastic Lymphoma

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