PB1755 URGENTLY TREAT A NEAR DEATH PATIENT OF CEREBRAL HERNIA CAUSED BY MYELOID SARCOMA
Authors: Zhou L. , Zhang X. , Feng S. , Hu X. , Zheng C. 
Published: June 2019

Background:

Intracranial myeloid sarcoma is a very rare disease presenting with or without bone marrow involvement. The optimal treatment remains unknown. Conventional AML‐like chemotherapy, radiotherapy, and surgery are the currently recommended strategies in controlling the disease progress.

Aims:

How to urgently treat a near death patient of cerebral hernia caused by myeloid sarcoma?

Methods:

A 28‐year‐old male patient was admitted to the hematological department at Anhui Provincial Hospital (China) in June 2017, with a 4‐day history of intense headache, worsening blurred vision and severe vomiting. The patient had a history of neurosurgical operation with resection of tumor involving the regions of torcular herophili and left transverse sinus in April 2017. The pathological findings of the tumor specimen led to a diagnosis of myeloid sarcoma. Postoperative cranial MRI indicated complete resection of the lesion. A diagnostic lumbar puncture showed no malignant cells, and methotrexate 10 mg, cytarabine 50 mg and dexamethasone 5 mg were given intrathecally. Bone marrow aspirate and biopsy had been conducted in May 2017, which demonstrated 5.5% blasts expressing CD13, CD33, CD34, CD117 and MPO, and the cytogenetic analysis demonstrated t(8;21)(q22;q22), and molecular studies showed a positive AML1/ETO rearrangement, but negative for c‐KIT, FLT3, NPM1, or CEBPA mutations. The diagnosis of acute myeloid leukemia (AML) was made. The patient did not receive any systemic chemotherapy.

Headache appeared on 60‐day after the operation, and aggravated gradually, accompanied by blurred vision and severe vomiting. Blood routine was normal at admission. After admission, the emergency cranial CT indicated a circular hyperdense mass (54mm × 37 mm), which was surrounded by hypodense peritumoral edema in the left cerebellar hemisphere, and the density of the lesions was uniform and the margin was clear.

The recurrence of intracranial myeloid sarcoma was considered. The patient could die at any time due to acute intracranial hypertension. He refused decompressive craniectomy and emergency cranial radiotherapy to alleviate the symptoms. Idarubicin (12 mg/m2.d × 3 days) combined with high‐dose cytarabine (2 g/m2 q12h × 3 days) was initiated for emergency chemotherapy. Mannitol and valproate were used to reduce intracranial pressure and prevent seizures.

Results:

Chemotherapy was very effective. On the second day of chemotherapy, the symptoms of headache were obviously relieved, the number of vomiting was reduced, and the vision gradually became clear. All of the above symptoms disappeared at the end of chemotherapy. On the first day after chemotherapy, the cranial CT indicated that the cranial lesion was markedly reduced (20mm × 15 mm), and on the sixth day after chemotherapy, the lesion was completely disappeared. The patient experienced 12 days of severe neutropenia. With the support of G‐CSF, the patient recovered with normal neutrophil on the fourteenth day after chemotherapy. He currently received high‐dose cytarabine (3 g/m2 q12h × 3 days) and prepared for hematopoietic stem cell transplantation.

Summary/Conclusion:

Standard dose of idarubicin (12 mg/m2 · d × 3 days) and high‐dose cytarabine (2∼ 3 g/m2q12h × 3 days) both can pass through the blood‐brain barrier, thus effectively controlling the progression of the disease. Our patient's treatment approach could provide a reference for the treatment of such disease in the future.

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