Search

Even though HIV is associated with worse prognosis in many malignancies, the clinical course of myelodysplastic syndrome (MDS) in HIV + patients has not been well studied. Determining the clinical presentation and outcomes of MDS in these patients would be important for future diagnostic strategies, as anemia and other cytopenias are commonly seen in HIV + patients. Unique data mining software was used to identify cases of MDS or AML in adult patients who were also HIV + at Albert Einstein/Montefiore Medical Center between 1 January 2003 and 1 January 2017. Using Chi-Square and Fisher's exact test, characteristics of the HIV + MDS patients were compared to 135 HIV - MDS patients from the same institution diagnosed between 1997 and 2011. Fourteen biopsy proven MDS patients were identified with concomitant HIV. HIV + MDS patients presented at a younger age (59 vs. 71 yrs, p  = .001) had higher risk disease, faster progression to acute leukemia, and worse overall survival (median survival 11.2 vs. 69.1 mo, p  < .001) compared to HIV - MDS controls. Additionally, there was a higher prevalence of clonal-hematopoiesis related mutations (ASXL1, DNMT3A) and a higher proportion of patients with high risk cytogenetics. Analysis of the largest single center cohort of HIV + MDS patients demonstrated that these individuals present at a significantly younger age and with higher risk disease than their HIV - counterparts.

Lung involvement has been reported in HTLV-1 carriers and in patients with ATLL. Whether there are differences in the pattern of lung involvement between ATLL and HTLV carriers in North American patients is unknown. We aimed to compare CT pulmonary findings among patients with HTLV-1 infection with and without ATLL. Among 140 patients with HTLV-1 diagnosis, 97 had CT chest available. Of these, 72 (74.2%) had ATLL and 25 (25.8%) did not have ATLL. CT chest abnormalities were present in 90 (92.8%) participants (94.4% in ATLL; 88% in non-ATLL). Higher rates of lymphadenopathy (69.4% versus 24%, p  < .01) and lower rates of bronchiectasis (25% versus 48%, p  = .04) were seen in ATLL compared to non-ATLL. Our study supports that staging of lung involvement in ATLL should consider HTLV-associated pulmonary findings as not all CT chest abnormalities necessarily represent malignant infiltration.

Progressive multifocal leukoencephalopathy (PML) is a life-threatening opportunistic infection of immunomodulatory therapies. PML cases reported in PubMed (1995-2017) following stem-cell transplantation (HSCT) or chemoimmunotherapy (CIT) for hematologic malignancies were reviewed. We found 107 cases, 40% were HSCT recipients (32 allogeneic, 11 autologous) and 40% indolent lymphomas receiving monoclonal antibodies (mAbs). HSCT cases had longer time to PML diagnosis (10.8 vs. 4 months, p < .001), higher proportion of PML therapy response (58% vs. 25%, p = .019), lower mortality rate (56% vs. 88%, p < .001), and longer median survival (8 vs. 2 months, p < .001). Outcome differences might be caused by selection bias as HSCT patients are most likely treated aggressively; however, time-dependent immune reconstitution might also contribute to their better prognosis. Increased use of mAbs and HSCT are associated with rising PML incidence in hematological malignancies, currently constituting the second largest vulnerable population after HIV-infected patients; further research is needed for its optimal treatment.

Lenalidomide (Len) is clinically indicated in myelodysplastic syndrome (MDS) but its use is limited by significant thrombocytopenia. Eltrombopag (EP) is a thrombopoietin receptor agonist that can stimulate platelet production and has shown preclinical efficacy in inhibiting leukemic cell proliferation. Thus, we determined the preclinical efficacy and safety of combining Len and EP in acute myelogenous leukemia (AML) and MDS. We found that single agent treatment of leukemia and lymphoma cell lines with EP and Len showed differential sensitivities to either agent. Combination therapy did not result in reversal of anti-malignant effects on these cells. Furthermore, the combination of Len and EP resulted in significant inhibitory effects on growth of leukemic colonies in the majority of primary MDS and AML samples. Most importantly, EP was able to reverse the anti-megakaryopoietic effects of Len in primary MDS patient samples. These results provide a preclinical rationale for the use of this combination in MDS and AML.

Eosinophilic variant of CML (eoCML) is a unique disease with a poor prognosis. Like the hypereosinophilic syndrome (HES), eoCML has no clinically identifiable reason for an increased eosinophil count in the peripheral blood. In contrast to HES, eoCML patients carry a distinct chromosomal abnormality. The bcr/abl fusion gene (Philadelphia chromosome) is the genetic basis of this clonal disease. Recently, eoCML has been separated from HES. Patients with eoCML frequently suffer organ damage including the heart and lungs. This damage is related to the release of eosinophilic granules in the blood, which results in fibrosis of the endothelial lining. We report a case of a peripheral vasculitis complicated by gangrene of the fingers in a patient with eoCML. Despite an almost complete response to CML treatment with Gleevac, combined with prednisone, aspirin and coumadin the patient sustained irreversible damage to the vascular lining of the distal arteries of the upper extremities.