Hairy Cell Leukemia in a Filipino Male during the COVID-19 Pandemic - Report of a Rare Case

Hairy cell leukemia (HCL) is a rare, chronic, mature B-cell lymphoproliferative disorder accounting for 2% of all leukemias. In this paper, we would like to present our experience in the management of HCL in a financially limited setting where other diagnostic tests and chemotherapy are unavailable. The case report aims to emphasize the recognition of the distinctive morphology of hairy cells in the peripheral blood in the consideration of the initial diagnosis. A 60-year-old Filipino male was incidentally found to have anemia, thrombocytopenia and an absolute neutrophilic count below 1,000 in a pre-operative clearance for elective herniorrhaphy. Blood smear revealed atypical lymphocytes with hair like cytoplasmic projections. CT-scan of the abdomen showed splenomegaly and prominent paraaortic nodes. Flow cytometry of the bone marrow aspirate was consistent with an involvement of a Mature B cell neoplasm markers CD19, CD20, CD22 and surface immunoglobulin lambda and hairy cell leukemia markers CD11c, CD103 and CD25. He responded to six-weekly sessions of Cladribine with remission of the bone marrow and hematologic parameters. HCL is a rare type of a mature B cell neoplasm characterized by pancytopenia, splenomegaly, bone marrow fibrosis and the presence of atypical lymphoid cells with hairy projections in blood, bone marrow and spleen. Immunophenotyping express CD11c, CD103, CD123, and CD25. BRAF V600E mutation is the disease defining genetic event. Cladribine and Pentostatin are the first line of treatment. Cases of leukemia can be easily overlooked because of the mild derangement in the complete blood count. A meticulous differential review of the atypical lymphocyte, is the first step in the diagnosis of this rare disease.Copyright © 2022, Philippine College of Physicians. All rights reserved.

IS PSORIATIC ARTHRITIS A RISK FACTOR for SEVERE COVID-19 INFECTION? DATA from the ARGENTINIAN REGISTRY SAR-COVID

Background: Comorbidities, particularly cardio-metabolic disorders, are highly prevalent in patients with psoriatic arthritis (PsA) and they were associated with an increased risk of atherosclerotic cardiovascular disease, which have been associated with higher morbidity and mortality. Whether PsA enhances the risk of SARS-CoV-2 infection or affects the disease outcome remains to be ascertained. Objectives: To describe the sociodemographic, clinical and treatment characteristics of patients with PsA with confrmed SARS-CoV-2 infection from the SAR-COVID registry and to identify the variables associated with poor COVID-19 outcomes, comparing them with those with rheumatoid arthritis (RA). Methods: Cross-sectional observational study including patients ≥18 years old, with diagnosis of PsA (CASPAR criteria) and RA (ACR/EULAR 2010 criteria), who had confrmed SARS-CoV-2 infection (RT-PCR or serology) from the SAR-COVID registry. Recruitment period was between August 13, 2020 and July 31, 2021. Sociodemographic variables, comorbidities, and treatments were analyzed. To assess the severity of the infection, the ordinal scale of the National Institute of Allergy and Infectious Diseases (NIAID)1 was used, and it was considered that a patient met the primary outcome, if they presented criteria of categories 5 or higher on the severity scale. For this analysis, Chi2 test, Fisher's test, Student's test or Wilcoxon test, and binomial logistic regression using NIAID>=5 as dependent variable were performed. Results: A total of 129 PsA patients and 808 with RA were included. Clinical characteristics are shown in Table 1. Regarding PsA treatment, 12.4% of PsA were receiving IL-17 inhibitors, 5.4% IL12-23 inhibitors, one patient apremilast and one abatacept. The frequency of NIAID≥5 was comparable between groups (PsA 19.5% vs RA 20.1%;p=0.976). (Figure 1). PsA patients with NIAID≥5 in comparison with NIAID<5 were older (58.6±11.4 vs 50±12.5;p=0.002), had more frequently hypertension (52.2% vs 23%;p=0.011) and dyslipidemia (39.1% vs 15%;p=0.017). In the multivariate analysis, age (OR 1.06;95% CI 1.02-1.11) was associated with a worse outcome of the COVID-19 (NIAID≥5) in patients with PsA, while those who received methotrexate (OR 0.34;95% CI 0.11-0.92) and biological DMARDs (OR 0.28;95% CI 0.09-0.78) had a better outcome. Conclusion: Although PsA patients have a higher frequency of cardiovascular and metabolic comorbidities than those with RA, the COVID-19 severity was similar. Most of the patients had mild SARS-CoV-2 infection and a low death rate.

MACROGLOBULINEMIA DE WALDENSTRÖM: RELATO DE CASO

Introdução: A macroglobulinemia de Waldenstrom (MW) é uma patologia linfoproliferativa neoplásica maligna das células plasmáticas e linfócitos B, normalmente responsável pela síntese das cadeias pesadas de imunoglobulinas. Diferente da leucemia linfoblástica aguda, os linfócitos mantêm a capacidade de se diferenciar e amadurecer em células plasmáticas. A MW possui características que variam de células linfoides maduras a plasmócitos. Imunofenotipagem de células obtidas a partir da medula óssea, dos linfonodos ou do sangue periférico de pacientes com essa doença mostram IgM citoplasmática detectável em células plasmáticas e imunoglobulina superficial na maioria dos linfócitos. Objetivo: Apresentar um caso de MW. Material e métodos: Revisão de prontuáriros médicos e literatura. Descrição do caso: Paciente do sexo masculino, 61 anos, apresentou epistaxe de grande volume com repetição, fadiga e astenia, além de perda de 15 quilos em 6 meses. Na admissão hospitalar apresentava os seguintes exames: anemia com hemoglobina 5,4 g/dL, hemácias de 1,45 milhão/mm3, plaquetopenia (87.000/mm3), presença de esplenomegalia importante, roleaux eritrocitário, esplenomegalia e linfonodomegalia abdominal. Solicitada avaliação com hematologista por paciente referir repetidas anemias ao longo da vida e histórico de transfusões de repetição. Seguem os resultados da investigação com especialisgta: Mielograma apresentou células linfoides de tamanho pequeno e aspecto maduro, algumas apresentando aspecto plasmocitoide. Imunofenotipagem de sangue periférico com CD20/CD38, CD38, CD200, CD19/CD200 positivo fraco;CD19, CD45, CD45/CD22, KAPPA, CD20: positivo de alta intensidade;KAPPA cito- plasmático e CD43 positivo. Dosagem de Imunoglobulinas: IgM 10.100 mg/dL, IgG 242 mg/dL e IgA 91,5 mg/dL. Pico monoclonal IgM/Lambda na imunofixação sérica. Exame de Coombs direto negativo. Como terapêutica, iniciou-se Ciclofosfamida associado a Rituximabe. O paciente evoluiu ao óbito devido ao Covid-19 durante a internação. Discussão: A MW é uma neoplasia rara, sendo que o paciente deste relato enquadra-se na idade média do diagnóstico de MW, que ocorre em torno dos 60 anos, maioria do sexo masculino. Grande parte dos pacientes apresenta sintomas como fadiga, fraqueza e sangramento (principalmente epistaxe). Outras manifestações como perda de peso, distúrbios visuais e fenômeno de Raynaud são menos comuns. Ao exame físico, encontra-se rotineiramente hepatoesplenomegalia e linfadenopatia. Proteinúria de Bence Jones está presente em um quarto de todos os pacientes com MW, devido a uma lesão predominantemente glomerular com depósitos de IgM e material amiloide. A função plaquetária está prejudicada pelo revestimento de plaquetas com IgM, e alguns pacientes podem apresentar defeitos da cascata de coagulação. Os níveis de IgM são marcadamente aumentados, e a crioglobulina pode ser detectada em alguns pacientes. O tratamento deve ser individualizado de acordo com as manifestações clínicas de cada paciente e seu curso clínico é variável. Causas de morte relacionam-se geralmente a hiperviscosidade, anemia, hemorragia, trombose e infecções, sendo que neste caso a infecção por Covid-19 atuou como fator definidor. Conclusão: É oportuno o relato de um caso de MW devido a raridade de seu diagnóstico e importância de estabelecer diagnóstico diferencial com doenças de maior prevalência.