key: cord-0690350-5zua0gsz authors: LAMURE, Sylvain; SALMANTON-GARCÍA, Jon; ROBIN-MARIETON, Elena; JAKSIC, Ozren; KOHN, Milena; MARCHESI, Francesco; MARCHETTI, Monia; EL-ASHWAH, Shaimaa; DEMIRKAN, Fatih; VALKOVIĆ, Toni; FERNÁNDEZ, Noemí; TISI, Maria Chiara; STOJANOSKI, Zlate; SEVAL, Guldane Cengiz; ILHAN, Osman; PREZIOSO, Lucia; MERELLI, Maria; LÓPEZ-GARCÍA, Alberto; LEDOUX, Marie-Pierre; KULASEKARARAJ, Austin; GONZÁLEZ-LÓPEZ, Tomás-José; GOMES DA SILVA, Maria; EMARAH, Ziad; DUARTE, Rafael F.; CATTANEO, Chiara; BLENNOW, Ola; BILGIN, Yavuz M.; BERGANTIM, Rui; BATINIĆ, Josip; CORDOBA, Raul; ESSAME, Jenna; NORDLANDER, Anna; NUNES RODRIGUES, Raquel; SACCHI, Maria Vittoria; ZOMPI, Sofia; BUSCA, Alessandro; CORRADINI, Paolo; HOENIGL, Martin; KLIMKO, Nikolai; KOEHLER, Philipp; PAGLIUCA, Antonio; PASSAMONTI, Francesco; DULÉRY, Rémy; CORNELY, Oliver A.; BESSON, Caroline; PAGANO, Livio title: COVID-19 and Hairy-Cell Leukemia: An EPICOVIDEHA Survey date: 2022-05-05 journal: Blood Adv DOI: 10.1182/bloodadvances.2022007357 sha: 67c235abf0673972ad07d4eee9bee26ff9a1275a doc_id: 690350 cord_uid: 5zua0gsz nan France, two patients (2%) had hairy cell leukemia (HCL) 9 . Furthermore recent single case 148 reports described either a concomitant diagnosis of COVID-19 and HCL 10 , and a critical 149 COVID-19 in a context of HCL 11 . In this study, we aim to report the characteristics and The 7 cases diagnosed less than one month before Covid-19 were considered as concomitant. 171 HCL was diagnosed more than one month before Covid-19 for 33 patients (83%) including 5 172 under frontline therapy (13%), 11 in remission (28%), 15 with untreated stable disease (35%), 173 one with a refractory disease (3%) and one with an unknown situation (3%). Regarding HCL 174 treatments received before Covid-19, 17 patients had received cladribine (43%) any time 175 including 16 as last treatment and three were exposed to rituximab (8%). Time between 176 cladribine treatment and COVID-19 was documented for 13 patients and ranged from 7 days 177 and 18 years (median 3 months); Eight of them had received Cladribine less than 6 months 178 before Covid-19. Due to the period covered by this study only 5 patients were vaccinated 179 against SARS-CoV-2 (13%) and variants were not documented. COVID-19 was severe for 19 180 cases (48%) and critical for 14 (35%). The median length of in-hospital stay was 15 days 181 (IQR: 8-26, range 1-84) and 8 patients were still hospitalized after 30 days (20%). Fourteen 182 patients (35%) were admitted in an intensive care unit (ICU), of whom 10 (24%) were 183 intubated, and the median stay at the ICU was 15 days (IQR: 6-30, range 1-57). A total of 10 184 patients died amounting to a 25% overall mortality, and of these 9 (90%) died due to the 185 COVID-19, one case for an undetermined reason. Among the 22 patients who were treatment-186 free for HCL, 5 had died (23%) which is similar to the proportion of 5 deaths out of 17 Hairy cell leukemia: present and future directions WHO Classification of Tumours of Haematopoietic and 291 Lymphoid Tissues Infections in hairy-cell leukemia Infectious complications in hairy cell leukemia Consensus guidelines for the diagnosis and management of 297 patients with classic hairy cell leukemia 2-chlorodeoxyadenosine 299 induces durable remissions and prolonged suppression of CD4+ lymphocyte counts in 300 patients with hairy cell leukemia Clinical and microbiological characteristics of the infections in 302 patients treated with rituximab for autoimmune and/or malignant hematological disorders Hairy cell leukemia and COVID-19 adaptation of treatment 305 guidelines Prolonged in-hospital stay and higher mortality after Covid-19 among 307 patients with non-Hodgkin lymphoma treated with B-cell depleting immunotherapy A Rare Case of Hairy Cell Leukemia with Unusual Loss of CD123 310 Associated with COVID-19 at the Time of Presentation Janus-faced course of COVID-19 infection in patients with 313 hematological malignancies EPICOVIDEHA: A Ready to Use Platform for 315 Epidemiological Studies in Hematological Patients With COVID-19 Characteristics of and Important Lessons From the 318 COVID-19) Outbreak in China: Summary of a Cases From the Chinese Center for Disease Control and Prevention COVID-19 infection in adult patients with hematological 322 malignancies: a European Hematology Association Survey (EPICOVIDEHA) Inherent and iatrogenic immune 325 defects in hairy cell leukemia: revisited Dexamethasone in Hospitalized Patients with Covid-19 Efficacy and Safety of the mRNA-1273 SARS-CoV-2 Vaccine Here we report the first cohort of patients with HCL and COVID-19. The incidence in the 204 survey is close to the previously reported 2% proportion of HCL among lymphoproliferative 205 neoplasm 2 . In the EPICOVIDEHA study, 64% of cases were severe/critical and the overall 206 mortality was 31%. The 83% of severe/critical patients reported here suggests a more severe 207 presentation of COVID-19, but the percentage of patients who died was comparable to the 208 entire EPICOVIDEHA cohort 14 . Former studies revealed a negative impact of anti-tumoral 209 treatment on infection outcomes 9 , however, in this study that included a limited number of 210 cases with variable treatment free period, we did not observe any effect of HCL treatment on 211 mortality, with the limitation of the heterogeneity of our study population concerning the 212 treatments received. The 20% of patients who stayed longer than 30 day in hospital suggest 213 the frequent persistence of COVID-19 as reported previously 9 . Besides, we observed a 214 significant proportion of new diagnoses of HCL, revealed by splenomegaly and/or cytopenia 215 in the situation of severe COVID-19, highlighting the importance of investigating cytopenia 216 when present at COVID-19 diagnosis. Actually, it is common for HCL patients to suffer from 217 atypical and severe life threatening infections since they present with complex immune 218 defects affecting both cellular and humoral mediated immunity 15 SARS-CoV-2, severe acute respiratory syndrome coronavirus 2 *asymptomatic is for patients without clinical signs or symptoms, mild for those with non-pneumonia and mild pneumonia, severe for those with dyspnoea, respiratory frequency ≥ 30 breaths per min, SpO2 ≤ 93%, PaO 2 /FiO 2 < 300, or lung infiltrates > 50% on tomography, and critical for patients admitted in intensive care for respiratory failure, septic shock, or multiple organ dysfunction or failure