Abstract The occurrence of acute leukemia during pregnancy is a rare pathologic situation and requires a multidisciplinary approach that includes at least a hematologist, gynecologist, neurologist, or psychotherapist and sometimes even intensive care unit care. The difficulties in caring for such a patient are many, from determining the risk associated with continuing the pregnancy, to choosing diagnostic tools and therapy appropriate for such a situation, to considering the patient's wishes and preferences. Below, we present the approach for cases of acute leukemia in pregnancy diagnosed and treated at the Emergency University Hospital of Bucharest (SUUB), Hematology Department, between January 1, 2017 and December 31, 2022, during a 6-year period.

Primary immune thrombocytopenia (ITP) is characterized by isolated low platelet count and it is a diagnosis of exclusion, contrasting to secondary ITP. Therefore, a positive diagnosis is difficult and requires extensive investigation. Some of the underlying conditions that are associated with ITP are lymphoproliferative disorders and infections, especially viral ones. In the present study, the case of a patient diagnosed with diffuse large B-cell lymphoma, who received chemotherapy and autologous hematopoietic stem cell transplantation is presented. After a complete remission of four years, the patient presented with sudden intense hemorrhagic syndrome and severely decreased platelet count. The most frequent causes of secondary ITP were excluded, including lymphoma relapse, and intravenous corticosteroids were started. However, shortly after hospital admission, the patient developed neuro-psychiatric anomalies, fever and pancytopenia, and West-Nile encephalitis was diagnosed. Although the initial development was favorable, he started to complain of progressive severe muscle weakness and eventually succumbed to infectious complications in the setting of prolonged hospitalization, corticotherapy, and immobilization.

Introduction Adult T-cell leukemia/lymphoma (ATL) is a peripheral T-cell neoplasm triggered by HTLV1 infection. According to the Japanese studies, the median onset of disease is around age 60 (Yamada Y et al. Br J of Haematol 2001, Kamiunten A et al. Hematol Oncol 2018). The aggressive forms exhibit a poor prognosis, due to intrinsic or acquired chemotherapy resistance and severe immunosuppression. Combination chemotherapy can improve the response rates, and consolidation with allogenic hematopoietic stem cell transplantation (allo-HSCT) prolongs complete remission (CR) (Yamada Y et al. Br J of Haematol 2001, Kamiunten A et al. Hematol Oncol 2018). The overall survival (OS) rates at 1 and 2 years after transplantation were reported to be 34% and respectively 27% (Kamiunten A et al. Hematol Oncol 2018). Romania is one of the regions with the highest prevalence of HTLV1 infection (5.33 at 10.000 donors in 2003-2008, Leperche S et al. Vox Sang 2009), therefore the prevalence of ATL is also higher. Methods A prospective observational study was performed on all patients with aggressive forms of ATL, diagnosed in Bucharest between April 2010 and January 2019, respectively 56 patients. Patients under 18 years of age and with chronic or smoldering type were excluded. The goal of this study was to better describe the disease characteristics and to discover novel factors that influence response and survival. All patients were assessed in collaboration with Necker Hospital, Paris, France, as reference center. Clinical and lab data were collected, the treatment was administered at investigator choice using CHOP/CHOP-like, Hyper-CVAD and LSG15 regimens, and allo-HSCT was offered to all eligible patients as soon as CR was obtained. Statistical analyses were performed using IBM SPSS Statistics 25. For survival analysis we used Kaplan-Meier estimate. The ROC curve was used to set cutoff values. The statistical significance was calculated using Chi-squared test and Fisher's exact test. Results In this study we analyzed a total of 56 ATL patients (M:F=1:1.3), with a median age of 42.5 years, all having aggressive types. Within this group, 33.9% had hypercalcemia and 73.2% elevated LDH (64.3% >2xN). Most used chemotherapy regimens were: CHOP/CHOP-like (60.7%), followed by LSG15 (23.2%) and Hyper-CVAD (7.1%); antiretroviral therapy (51.8%) and allo-HSCT (12.5%) were also used. LSG15 regimen is not fully applicable in our country due to unavailability of Ranimustine and Vindesine, therefore a small proportion of patients received this treatment. In multivariate analysis, factors showing significant association with lower rate of CR were: age >40.5 years, leukocytosis with a cut-off of 8950/μl, hypercalcemia >9.5 mg/dl, high LDH >512 U/L. LSG15 was associated with a higher CR rate (50%) compared to CHOP/CHOP-like (22.7%) and Hyper-CVAD (33.3%), although not statistically significant. Median OS was 6.5 months (5.1 - acute type, 8.0 - lymphomatous type), versus 3.5 months in acute type and 9.5 months in lymphomatous type in previous data in our center. Early death rate (< 1 month) was found in 8.9% (n=5) of patients. Factors associated with early death were: acute type (n=4), leukocytosis (n=5), hypercalcemia (n=4), high LDH (n=5), extranodal involvement (n=4). Patients who obtained CR (n=9) had a better survival that those who did not (median OS 40.7 vs 5.1 months, p=0.000) (Fig a); undergoing allo-HSCT also significantly increased OS (median OS not reached vs 6.1 months, p=0.003) (Fig b). Among patients who underwent allo-HSCT, OS at 1 year and 2 years after the allo-HSCT were 57.1% and 42.9%, respectively. Three patients died, all less than 1 year after transplantation. Conclusions Median age at diagnosis in our group was lower (42.5 years) versus the Japanese studies (Yamada Y et al. Br J of Haematol 2001, Kamiunten A et al. Hematol Oncol 2018). This might reflect an origin of infection in the 1980s in Romania, where other outbreaks occurred by horizontal transmission in that period, including an outbreak of HIV-1 (Patrascu I V et al, Lancet 1990). Future in-depth studies of this cohort might better correlate age of infection, HTLV1 subtype and latency to ATL. The best predictors of better survival were obtaining CR and receiving allo-HSCT. Among the treatments we used, LSG15 regimen had higher rate of CR. Survival after transplantation was higher in our group compared to the Japanese studies (Kamiunten A et al. Hematol Oncol 2018). Disclosures Constantinescu: AgenDix GmbH: Other: Co-Founder, MyeloPro Research and Diagnostics; AlsaTech: Other: Co-Founde; Wiley & Sons: Other: Editor in Chief, Journal of Cellular and Molecular Medicine; Novartis: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau. Hermine:Celgene: Research Funding; AB science: Consultancy, Equity Ownership, Honoraria, Research Funding; Novartis: Research Funding.

Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare disease that is frequently underdiagnosed due to clinical features that are similar to those of non-Hodgkin lymphomas, systemic lupus erythematosus (SLE), or infectious reactive lymphadenopathy. An excisional biopsy is required. We report a young Caucasian female diagnosed with KFD with skin lesions, complicating with SLE. The clinical course, laboratory, and CT findings are described, as are histopathologic features, for a better recognition of this rare disorder in clinical practice.

POEMS syndrome (acronym consisting of: polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) is an uncommon disorder associated with an underlying plasma cell dyscrasia. There is no single specific test for POEMS, and due to its rarity and heterogeneity, patients are often mis- or underdiagnosed. Castleman disease (CD) is a rare lymphoproliferative disorder, closely related to POEMS syndrome; ~11%-30% of POEMS patients are associated with concomitant CD. In contrast to frequently published reports on vascular events in POEMS syndrome affecting coronary arteries or lower limbs, cases of cerebrovascular events are rarely mentioned in literature. We hereby report a patient with POEMS syndrome accompanied by CD who presented recurrent strokes and splenic infarction.

ABSTRACTBACKGROUNDPeripheral T and natural killer (NK) cell lymphomas represent a heterogeneous group of diseases with varied clinical features, prognosis and response to treatment.Content and comments: Flow cytometric immunophenotyping facilitated the diagnosis and classification for T/NK-cell neoplasms, and is very useful in the identification of therapeutic target markers. Clinical, immunophenotypic, histopathological, immunohistochemical, molecular and genetic methods must be correlated because none of them is strong enough alone for diagnosis.CONCLUSIONThe low incidence of NK/T-cell lymphoma poses real difficulties for a complete and correct assessment. The unspecified lymphomas represent a heterogeneous group, which requires additional studies to elucidate their biological and genetic bases, to separate them. The group of NK/T-cell lymphomas remains a challenge for researchers.

We present the case of a 53 years old woman diagnosed with splenic marginal zone lymphomas with plasmacytic differentiation (after a lymph node biopsy), who, complained of mild asthenia, weight loss (about 10 kg in 9 months), spatial disorientation during the last period. The clinical examination revealed slight pallor, normal cardiovascular and respiratory examination; painful cervical, about 5cm in diameter and also non-painful inguinal lymphadenopathies, increased consistency, freely movable, about 2 cm in diameter. The patient presented enlarged liver (lower limit at 3 cm below the ribs) and spleen (inferior pole at the ombilicus). The laboratory tests showed leucocytosis with lymphocytosis-a clonal population of lymphocytes- CD19+, CD20low+, CD22+, CD5low+, CD24+, CD200low+, CD79B+, CD43-, FMC7+/-, CD10+/-, CD34-, BCL2+, TdT-, CD34-, CD10-, CD3-. We suggested the diagnosis of mantle cell lymphoma, blastoid variant and performed a bone marrow biopsy . The bone marrow biopsy excluded the diagnosis of mantel cell lymphoma, based on the absence of cycline D1. The histopathological appearance and the immunohistochemical tests (CD20+, CD79a+, CD5low+, TdT-, CD34-cycline D1-) suggested a blastoid variant of small lymphocytic lymphoma.

Chronic myeloid leukemia (CML) is a hematopoietic stem cell disorder in which the diagnosis is confirmed by detection of a genetic marker: Philadelphia (Ph) chromosome in almost 90% of cases. Some of Ph1 negative patients, nevertheless, test positive for the abnormal gene, or the abnormal protein associated with this chromosome, when more sensitive studies, such as PCR or FISH are used and nowadays the diagnosis of CML is based, not only on cytogenetic, but also on molecular analysis. The better understanding of the CML biology provided by the latest researches requires a deeper knowledge about the epidemiologic data in each geographic area, so the compiling of a National and/or European Registry for CML patients, that represents one of the aims of this study, became a stringent matter in our days; it can offer valuable data concerning the real incidence of this disease in Romania and can provide the basics for establishing long-term budgetary strategies.