Rare-55. langerhans cell histiocytosis (lch) associated with leptomeningeal acute myeloid leukemia (aml): a case report

We describe a 59-year-old patient with LCH of the right frontal sinus metastatic to regional lymph nodes and colon who presented with acute meningoencephalitis and was found to have AML isolated to the CNS. The patient initially presented with antibiotic-refractory recurrent sinusitis and was diagnosed with LCH following sinus biopsy. He was treated with 2 cycles of cytarabine (100 mg/m2 daily x 5 days) but progressed with metastatic disease to lymph nodes and colon (biopsy confirmed). He received 2 cycles of clofarabine with partial response on CT/PET, which was complicated by neutropenia (WBC 0.95, ANC 0.47). He was admitted to the hospital with altered mental status, generalized weakness, and multiple cranial neuropathies. Work up revealed a right frontal dural based mass and cerebrospinal fluid analysis significant for protein >550 mg/dL, glucose <20 mg/dL, and 2105 nucleated cells/mcL. CSF cultures were negative and he did not respond to empiric antibiotics. CSF flow cytometry was consistent with AML, monocytic subtype. Analysis of peripheral blood was notable for pancytopenia without blasts or clonal population of cells. Due to pancytopenia and poor performance status the patient was unable to receive chemotherapy and died on hospice shortly after diagnosis. To the best of our knowledge, this is the only reported case of central nervous systemic AML associated with LCH.
Source: Neuro-Oncology - Category: Cancer & Oncology Authors: Tags: RARE TUMORS Source Type: research