Objective Myeloid/lymphoid neoplasms with fibroblast growth factor receptor-1 (FGFR1) rearrangement are hematopoietic stem cell disorders with a poor prognosis, but no established standard therapy. Methods We experienced a patient with T-lymphoblastic lymphoma (LBL) associated with FGFR1 rearrangement who underwent cord blood transplantation, but died of pulmonary complication. We collected the clinical data of patients with FGFR1 rearrangement from the medical literature and analyzed 45 patients, including our patient. Results The primary diagnoses were myeloproliferative neoplasm (MPN) or myelodysplastic syndromes (MDS) in 14 and acute leukemia or LBL in 31. In MPN and MDS patients, the cumulative incidence of transformation to blast phase (BP) at 12 months was 46.2%. The 1-year overall survival (OS) from diagnosis in all cases was 43.1%. With regard to the impact of treatment response on survival, the achievement of complete response with a landmark at 2 months after diagnosis of BP was associated with a superior OS (40.0% vs. 26.0% P = 0.011 for 1-year OS from BP). Allogeneic hematopoietic stem cell transplantation (HSCT) was performed in 13 patients, and the 1-year OS from allogeneic HSCT was 61.5%. The hazard ratio for mortality was 0.34 (95% CI, 0.08-1.51, P = 0.15) for allogeneic HSCT treated as a time-dependent covariate, which suggests that allogeneic HSCT may confer a clinical benefit. Conclusion The further accumulation of clinical data is needed to determine the optimal therapeutic approach for these neoplasms.

https://www.tandfonline.com/doi/pdf/10.1080/10245332.2018.1446279

Clinical outcomes of myeloid/lymphoid neoplasms with fibroblast growth factor receptor-1 (FGFR1) rearrangement.

Distinguishing brain inflammation from grade II glioma in population without contrast enhancement: a radiomics analysis based on conventional MRI.

Numerous non-neoplastic and neoplastic conditions manifest with distinctive features in blood. Although automated complete blood count (CBC) data are essential, CBC information alone is insufficient for diagnosis. Consequently, morphologic review of blood smears is still relevant in the era of sophisticated automated analyzer systems. Pathologist interpretation of the peripheral blood smear, in conjunction with CBC and clinical information, can provide rapid diagnostic information and guide cost-effective targeted laboratory testing. Pathologist review of blood smears can be used to diagnose cases in which the clinical findings are misleading or nonspecific. Here, we discuss a selection of cases in which the pathologist examination of the blood smear can aid in rapid and accurate diagnosis and guide appropriate treatment. Exemplary non-neoplastic disorders with distinctive morphologic blood features of RBC, neutrophils, monocytes, and lymphocytes will be highlighted. The differential diagnostic considerations in blood smears with RBC destruction will be presented, expanding beyond microangiopathic hemolytic anemia. Lymphocyte morphologic assessment guides differential diagnosis including the identification of rare germline disorders. In each case presented, the integration of morphologic features guided additional testing with confirmation of the diagnosis. Peripheral blood smear review is timely and cost effective, even in an era of sophisticated automated laboratory testing.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/ijlh.12667

Does morphology matter in 2017? An approach to morphologic clues in non‐neoplastic blood and bone marrow disorders

Objectives: The 8p11 myeloproliferative syndrome (EMS) is an extremely rare, generally aggressive haematologic malignancies. This study provided the clinical outcomes and therapeutic strategies for...

https://www.tandfonline.com/doi/pdf/10.1080/16078454.2020.1854493

Myeloid/lymphoid neoplasm with CEP110-FGFR1 fusion: An analysis of 16 cases show common features and poor prognosis.

Herpes Simplex Encephalitis Initially Erroneously Diagnosed as Glioma of the Cerebellum: Case Report and Literature Review.

The 8p11 eosinophilic myeloproliferative syndrome (EMS) is an aggressive neoplasm driven by translocation of the fibroblast growth factor receptor 1 and often transforms to leukemias and lymphomas that are refractory to treatment. The first case was identified in 1983, and to date over 70 cases have been reported in the literature. Despite those reports, no consensus exists on management of this condition, and inconsistency in treatment regimens is even more pronounced in the pediatric literature. We report a case of a male infant with the 8p11 EMS, review the published pediatric experience with EMS, and discuss treatment strategies for this enigmatic hematological disorder.

Naresh Reddivalla

Papers

Pediatric 8p11 eosinophilic myeloproliferative syndrome (EMS): A case report and review of the literature

Anaplastic astrocytoma mimicking herpes simplex encephalitis in 13-year old girl.

Florid erythrophagocytosis by neutrophils in peripheral blood.