Mangaonkar, Abhishek A.

7058 Background: The 2016 WHO classification includes myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN-U), as an MDS/MPN overlap syndrome not meeting criteria for well-defined entities such as CMML. No standard prognostication or treatment guidelines exist for such patients. Methods: We retrospectively identified MDS/MPN-U cases from 1990-2016 through our myeloid malignancies database. All bone marrow reports were reviewed to ensure compliance with 2016 WHO criteria. Clinical & cytogenetic parameters at diagnosis were assessed & compared with treatment outcomes. Results: Eighty nine patients met study criteria, with a median age of 69 years (range: 37-93); 58 (65%) males. Median follow-up was 22.2 months (range: 0-172), with 41 (46%) deaths & 13 (15%) leukemic transformations. Median OS was 24.8 months (range: 0-172). 43 (53%) patients had an abnormal karyotype, with common abnormalities being trisomy 8 (12%), complex karyotype (9%) & del (20q) (6%). Given the fewer types of abnormalities identified, the IPSS cytogenetic stratification was more effective than IPSS-R, with risk categorization including; 45 good (55%), 20 intermediate (25%) & 16 high risk (20%) respectively (8 unavailable). On univariate analysis, increased age (p = 0.05), decreased hemoglobin (p = 0.02), higher ANC (p = 0.03), circulating immature myeloid cells (p = 0.02), higher LDH (p = 0.009), absence of bone marrow ring sideroblasts (p = 0.001) & higher risk (intermediate & high) IPSS cytogenetic categories (p = 0.01) adversely impacted OS. In a multivariate model that included the aforementioned variables, higher risk IPSS cytogenetics retained a negative prognostic impact (p = 0.04). 28 patients received a median of 6 cycles (range: 1-21) of hypomethylating agent therapy (HMA), with an overall response rate of 18% (CR-3, PR-2). All responders had an abnormal karyotype (p = 0.01). However, HMA did not affect either OS or LFS. Conclusions: Intermediate & high risk IPSS cytogenetic categories independently & adversely impact survival in WHO defined MDS/MPN-U patients. HMA use did not impact OS; however, patients with abnormal karyotypes were more likely to respond.

Background/Aims. Autonomic dysfunction (AD) after allogeneic stem cell transplant (SCT) is a rare occurrence and likely immune-mediated in etiology. There is limited literature on this topic and hence, we wish to briefly describe management of two cases at our institution and their outcomes. Methods. We retrospectively identified two patients with immune-mediated AD after SCT from our database. Immune-mediated AD was defined as AD secondary to an immune-mediated etiology without an alternative cause and responding to immunosuppression. Results. The first case is of a 32-year-old man with acute myeloid leukemia (AML) who underwent double umbilical cord allogeneic SCT. The second patient was a 51-year-old woman with secondary AML who underwent matched-related donor allogeneic SCT. Both underwent an extensive work-up for an underlying etiology prior to treatment with intravenous immunoglobulin (IVIG). Conclusions. AD after SCT is a rare yet significant clinical entity. A work-up of underlying etiology should be performed. IVIG is a treatment option for these patients.

In this article, we present the case of a 52-year-old male with a history of parotid carcinosarcoma with initial diagnosis being 18 months prior. Initial treatment included a combination of gamma knife surgery coupled with high dosage chemotherapy and X-ray radiation therapy. At the time of follow-up, the patient presented with no complaints and had a nearly normal physical exam with the exception of some facial nerve weakness on the same side as the initial surgery. Despite being asymptomatic, the patient had a significant progression of disease that was manifested with intracranial lesions, multiple pathologic fractures, and a dramatic increase in overall tumor burden. Ultimately, the patient decided to pursue comfort measures only and succumbed to the disease peacefully soon thereafter.