Paraplegia as the presenting manifestation of extramedullary megakaryoblastic transformation of previously undiagnosed chronic myelogenous leukemia

Barbara Bryant, Jack B. Alperin, M. Tarek Elghetany

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Extramedullary tumors, also known as granulocytic sarcomas (GS), occur most frequently in acute myelogenous leukemia (AML). They may signal the onset of the accelerated phase of chronic myelogenous leukemia (CML) or the blastic transformation of a myeloproliferative disorder. Occasionally, a GS may be the presenting sign of undiagnosed AML, and rarely the presenting sign of undiagnosed CML or aleukemic leukemia. Paraplegia due to a spinal cord GS is an extremely rare presentation of undiagnosed leukemia. This is the first case report of paraplegia as the presenting manifestation of extramedullary megakaryoblastic transformation of previously undiagnosed CML. A 53-year-old woman reported back pain for 6 days, rapidly progressing to paraplegia. Physical examination noted a large abdominal mass and flaccid paralysis in both lower extremities. Spinal MRI revealed a T4-T6 vertebral mass causing spinal stenosis and cord compression. Tumor debulking and laminectomy were performed emergently. The tumor consisted of noncohesive blast cells. The CBC revealed a leukocyte count of 238,300/μl and a differential consistent with CML. Reexamination of the patient found that the abdominal mass was a giant spleen. Further immunohistochemical studies of the tumor were consistent with extramedullary acute megakaryoblastic blast transformation of CML. Although extramedullary blast crises herald the accelerated phases in ∼10% of CML cases, megakaryoblastic blast transformation of CML accounts for less than 3% of these cases. The combination of acute paraplegia and megakaryoblastic transformation in a previously undiagnosed patient with CML is extremely rare and may pose a diagnostic dilemma.

Original languageEnglish (US)
Pages (from-to)150-154
Number of pages5
JournalAmerican Journal of Hematology
Volume82
Issue number2
DOIs
StatePublished - Feb 2007

Fingerprint

Paraplegia
Leukemia, Myelogenous, Chronic, BCR-ABL Positive
Myeloid Sarcoma
Lymphocyte Activation
Acute Myeloid Leukemia
Neoplasms
Leukemia
Leukemia, Myeloid, Chronic Phase
Blast Crisis
Spinal Stenosis
Myeloproliferative Disorders
Spinal Cord Compression
Laminectomy
Back Pain
Leukocyte Count
Paralysis
Physical Examination
Lower Extremity
Spinal Cord
Spleen

Keywords

  • Chronic myelogenous leukemia
  • Granulocytic sarcoma
  • Megakaryocytic leukemia
  • Paraplegia

ASJC Scopus subject areas

  • Hematology

Cite this

Paraplegia as the presenting manifestation of extramedullary megakaryoblastic transformation of previously undiagnosed chronic myelogenous leukemia. / Bryant, Barbara; Alperin, Jack B.; Elghetany, M. Tarek.

In: American Journal of Hematology, Vol. 82, No. 2, 02.2007, p. 150-154.

Research output: Contribution to journalArticle

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abstract = "Extramedullary tumors, also known as granulocytic sarcomas (GS), occur most frequently in acute myelogenous leukemia (AML). They may signal the onset of the accelerated phase of chronic myelogenous leukemia (CML) or the blastic transformation of a myeloproliferative disorder. Occasionally, a GS may be the presenting sign of undiagnosed AML, and rarely the presenting sign of undiagnosed CML or aleukemic leukemia. Paraplegia due to a spinal cord GS is an extremely rare presentation of undiagnosed leukemia. This is the first case report of paraplegia as the presenting manifestation of extramedullary megakaryoblastic transformation of previously undiagnosed CML. A 53-year-old woman reported back pain for 6 days, rapidly progressing to paraplegia. Physical examination noted a large abdominal mass and flaccid paralysis in both lower extremities. Spinal MRI revealed a T4-T6 vertebral mass causing spinal stenosis and cord compression. Tumor debulking and laminectomy were performed emergently. The tumor consisted of noncohesive blast cells. The CBC revealed a leukocyte count of 238,300/μl and a differential consistent with CML. Reexamination of the patient found that the abdominal mass was a giant spleen. Further immunohistochemical studies of the tumor were consistent with extramedullary acute megakaryoblastic blast transformation of CML. Although extramedullary blast crises herald the accelerated phases in ∼10{\%} of CML cases, megakaryoblastic blast transformation of CML accounts for less than 3{\%} of these cases. The combination of acute paraplegia and megakaryoblastic transformation in a previously undiagnosed patient with CML is extremely rare and may pose a diagnostic dilemma.",
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