Abstracts Clinical Lymphoma, Myeloma & Leukemia September 2023 S380 MPN-073 Development of a Diagnostic Algorithm for the Early Identification of Patients With Systemic Mastocytosis George Dranitsaris PhD1,2, Dakota Powell MPH3, Heather Neuhalfen MBA1, Aaron Peevyhouse MS1, Kerri Miller PharmD3, Teresa Green MSPH3, Tara Graff MD4 1Quality Cancer Care Alliance, Tacoma, USA. 2Syracuse University, Syracuse, USA. 3Blueprint Medicines, Cambridge, USA. 4Mission Cancer and Blood, Des Moines, USA Background: Systemic mastocytosis (SM) is a rare hematological disorder characterized by the accumulation and activation of mast cells in various tissues and organs of the body, including the skin, bone marrow, liver, spleen, and gastrointestinal tract. The excessive and uncontrolled activation of mast cells can lead to a wide range of symptoms, including skin lesions, flushing, itching, abdominal pain, diarrhea, nausea, and muscle weakness. Given the heterogeneous clinical presentation, part of the challenge in the effective management of SM patients is timely diagnosis. In this study, the development of a diagnostic (Dx) algorithm, or tool, for the early detection of SM is described. Methods: Data from 105 SM patients (50 indolent SM, 32 advanced SM, 23 SM type undocumented) was collected from QCCA, a network of 19 community hematology practices from across the United States. Data collection on a second sample of 104 non-SM control patients diagnosed with blood cancers was also undertaken. General linear models (GLM) with a logit link function were used in a backwards elimination process with the P-value set at < 0.05 to identify patient factors at presentation that were associated with a diagnosis of SM. A Dx scoring algorithm (range: 0-26) was then derived from the final model coefficients, with the intent of correctly differentiating between patients with and without SM. A receiver operating characteristic (ROC) curve analysis was then done to measure the Dx accuracy of the algorithm. Results: Seven SM Dx factors were identified and these included patient age less than 60, lymph node status, absolute neutrophil count, diarrhea, rash, skin lesions, and unintended weight loss at presentation. The ROC analysis indicated good predictive accuracy with an area under the curve of 0.89 (95% CI 0.84–0.93). At presentation, patients with a total score of ≥ 9 units would be considered at high risk for a positive SM diagnosis, with the associated sensitivity, specificity, and positive predictive value being 84.8%, 76.0%, and 82.7%, respectively. Conclusions: The clinical application and planned external validation of this Dx tool will support the early diagnosis of SM patients, which should facilitate the timely initiation of effective therapies. Keywords: MPN, systemic mastocytosis, diagnosis, predictive factors, algorithm MPN-082 Myeloproliferative Neoplasm Confronts Lymphoproliferative Disorder: A Case Report Mona Taalab MD, PhD1, Marwa Zaki MD, PhD2 1Internal Medicine Department, Faculty of Medicine, Mansoura University, Mansoura City, Egypt. 2Pathology Department, Faculty of Medicine, Mansoura University, Mansoura City, Egypt Context: Essential thrombocythemia (ET) is a BCR-ABL1negative myeloproliferative neoplasm (MPN) that may evolve into myelofibrosis. Vascular events and leukemic transformations are major complications of such neoplasm; however, these MPNs are unlikely to be complicated with malignant lymphoma. Objective: We report on a post-essential thrombocythemia myelofibrosis (PET-MF) patient with concurrent non-Hodgkin lymphoma (NHL). Patient: A 53-year-old male presented with bilateral enlarged upper neck swellings, desperately treated with antibiotics and anti-inflammatory drugs. A few weeks later, he developed progressive anemia associated with low-grade night fever and drenching sweats. Afterward, he received 2 units of packed red blood cells before referral to our hospital for further management. Interventions: General examination revealed multiple bilateral upper cervical, parotid, and inguinal lymph nodes (LN) that were non-tender and firm in consistency; the largest was 3.2×3.5 cm. Abdominal examination showed splenomegaly 8 cm below the left costal margin. Complete blood count showed the following: white blood cell count: 13.4×109/l, absolute neutrophil count: 10.3×109/l, hemoglobin: 11.5 g/dl, mean corpuscular volume: 84.7 fl, platelets: 753×109/l, reticulocytes count 5%, total bilirubin: 2.8 mg/dl, direct bilirubin: 0.4 mg\dl, lactate dehydrogenase: 612U/L, and positive +2 direct antiglobulin test (DAT). Main Outcome Measures: Right inguinal LN biopsy revealed NHL, diffuse large B-cell type. Bone marrow examination was done in conjunction with LN biopsy for staging and to figure out the cause of thrombocytosis. It showed 60% cellularity, megakaryocytes present in clusters, and clumps with atypical forms. Silver stain displayed grade 2 myelofibrosis. The peripheral blood examination was negative for JAK2 and MPL, but positive for CALR. Results: The final diagnosis was consistent with (PET-MF) with concurrent diffuse large B-cell type NHL and secondary autoimmune hemolytic anemia (AIHA). Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) therapy was planned; however, the patient developed hyperuricemic nephropathy and, while admitted for hydration and uricosurics treatment, contracted a COVID-19 infection 5 days following the hospital admission. He passed away 10 days later because of type I respiratory failure. Conclusions: PET-MF is a unique disease in mutational profile and overall survival. Coexistence with NHL is rare. Simultaneous diagnosis is challenging for optimal disease management. Keywords: MPN, PET-MF, NHL, AIHA, case MPN-091 Indirect Treatment Comparison of Momelotinib vs Fedratinib Safety in Patients With Myelofibrosis Lucia Masarova MD1, Francesca Palandri MD, PhD2, Srdan Verstovsek MD, PhD1, Ruben Mesa MD3, Claire Harrison MD, FRCP, PRCPath4, Gautam Sajeev ScD5, Boris Gorsh PharmD6, Ryan Simpson PhD5, Sang Cho
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