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First described in 1985, intermediate cell histiocytosis is a rare disorder of the cutaneous dendritic cell group with a varied clinical presentation and evolution. The pathologic substrate is constituted by the proliferation of indeterminate cells (ICs) that are immunophenotypically characterized by the positivity of CD1a, CD68, and faint/focal S100, plus the negativity for CD207 (langerin). The authors present the case of a healthy elderly woman who presented generalized dome-shaped reddish cutaneous nodules over her trunk, neck, face, and extremities over a period of 18 months. A laboratory and imaging work-up ruled out internal involvement. The skin biopsy was consistent with IC histiocytosis. The patient was treated with narrowband ultraviolet B phototherapy, which resulted in an excellent short-term outcome

Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma comprising a heterogeneous group of disorders with variable histological and clinical behavior. Although other lymphomas may present in the leukemic phase more frequently, this appearance is unusually observed among DLBCL cases. Diagnosing lymphoma is not always easy, and the patient’s clinical status quite often may hamper invasive procedures for diagnosis pushing the clinician to look for alternatives to reach the nearest possible accurate diagnosis. The authors report the case of a middle-aged man who presented the history of malaise, weight loss, and low-grade fever. The peripheral blood count showed leukocytosis with the presence of blasts and thrombocytopenia. The cytological morphology and immunophenotyping of the peripheral blood and bone marrow aspirate, as well as the bone marrow biopsy accompanied by a thorough immunohistochemical analysis, rendered the diagnosis of DLBCL in the leukemic phase. The patient was prescribed R-CHOP with a favorable outcome. Intra-abdominal lymph node biopsy was avoided because of the patient’s critical medical condition. The authors highlight this rare form of presentation of DLBCL as well as the combination of peripheral blood, bone marrow aspirate, and bone marrow biopsy for reaching the diagnosis in cases were a lymph node sample is unavailable for the diagnostic work-up

IntroductionKimura’s disease (KD) is a rare chronic inflammatory disorder characterized by subcutaneous lymphoid hyperplasia with peripheral eosinophilia. Kidney involvement is reported in 15%–18% of adult patients with KD, in many cases as nephrotic syndrome. We present a case of overlapping membranous nephropathy and IgA nephropathy associated with KD.Case reportA 27-year-old man was admitted with a history of bilateral leg edema for the last 2 months and concomitant progressive increase of cervical mass and fever. Laboratory findings were as follows: peripheral leukocyte count, 10,080/mm³; eosinophils, 3,200/mm³ (31.7%); serum creatinine, 0.83 mg/dL; and eGFR: 140 mL/min per 1.73 m2. Urinalysis revealed the presence of hematuria and proteinuria and the following results: 24-h proteinuria, 12.9 g; serum albumin, 1.3 g/dL; and elevated IgE level, 750 kU/L. Serologies for hepatitis B, hepatitis C, HIV, and VDRL were all negative. Complement C3 and C4 levels were normal. No monoclonal protein was detected in blood and urine. Parasite infestation was discarded. A biopsy of the cervical lymph node revealed eosinophilic lymphoid hyperplasia, suggesting KD. A kidney biopsy revealed findings consistent with the overlapping of membranous nephropathy with IgA nephropathy. The patient was treated for KD with prednisone 1 mg/kg/d with progressive dose tapering and posterior association of methotrexate 15 mg/week. A renin–angiotensin system inhibitor was prescribed for nephrotic syndrome. The cervical mass regressed, and proteinuria achieved partial remission, with an increase in serum albumin level and normalization of eosinophils and IgE levels.ConclusionAlthough uncommon, kidney involvement must be considered in patients with KD. Glomerular diseases are the most frequent form of kidney injury.

Introdução: a LMC é uma doença mieloproliferativa que afeta principalmente adultos entre 50 e 55 anos. No Brasil, as informações do Sistema de Informações Ambulatoriais do SUS apontam que, em 2017, 12.531 pacientes tiveram APAC aprovada para o tratamento da LMC.O monitoramento da doença por meio da avaliação da resposta molecular é fundamental para o cuidado do paciente com LMC. O PCR quantitativo (reação em cadeia da polimerase em tempo real) fornece parâmetros adequados de avaliação para que o profissional de saúde possa intervir nos momentos corretos de forma a reduzir a chance de progressão da doença, provendo o melhor desfecho ao paciente, incluindo a possibilidade de descontinuação do tratamento em pacientes elegíveis. Apesar do exame constar no Protocolo Clínico e Diretrizes Terapêuticas (PCDT) da LMC, não é possível realizar o monitoramento da resposta molecular pelo SUS visto que o teste não possui reembolso. Objetivo: obter recomendações de especialistas sobre a importância, financiamento e reembolso do monitoramento molecular no SUS. Métodos: seis especialistas em LMC com diferentes perspectivas participaram do painel. A discussão foi baseada utilizando as principais publicações sobre o exame de PCR quantitativo no monitoramento da LMC. Resultados: recomendações dos especialistas: 1) O monitoramento molecular deve fazer parte do tratamento integral dos pacientes com LMC para reduzir as chances de progressão da doença e custos ao sistema de saúde; 2) O governo deveria colocar em prática o que está preconizado no PCDT da LMC no Brasil: realização do monitoramento da resposta molecular via PCR quantitativo; 3) O governo deveria criar um código com nomenclatura e valor de reembolso adequados no SIGTAP, para que o teste passe a ser realizado e coberto pela rede pública de saúde, já que o mesmo consta no PCDT da doença e a APAC existente não cobre os seus custos operacionais para realização; 4) Os pacientes com LMC em fase crônica devem realizar um PCR quantitativo a cada 3 meses e após atingir a Resposta Molecular Maior (0,1% de BCR-ABL1 pela escala internacional) a cada 6 meses, conforme preconizado no PCDT; 5) Os pacientes devem ser monitorados em laboratórios de referência que são padronizados de acordo com a escala internacional; 6) Os laboratórios que estão dentro dos centros públicos de referência conseguem absorver toda a demanda de testes do Brasil. Mais centros poderiam ser habilitados através de uma acreditação da ABHH; 7) O monitoramento molecular adequado poderá permitir que alguns pacientes parem de tomar os medicamentos e, por consequência, autofinancie o teste molecular para todos os pacientes do SUS. Conclusão: É urgente encontrar uma solução de financiamento do teste molecular (BCR-ABL) para garantir o monitoramento dos pacientes com LMC no SUS. A economia que pode ser gerada com pacientes que param de tomar a medicação quando monitorados poderá garantir o financiamento do exame, conforme preconizado no PCDT.

Introduction: Chronic Myeloid Leukemia (CML) is a myeloproliferative disease that affects mainly adults between 50 and 55 years. In Brazil, information from the Sistema Único de Saúde (SUS) Outpatient Information System indicates that 12,531 patients had the Autorização de Procedimento Ambulatorial (APAC) approved for the CML treatment in 2017. Disease monitoring through molecular response evaluation is critical to the care of CML patients. The quantitative PCR test (real-time polymerase chain reaction) provides adequate evaluation parameters that allow the health professional to intervene at the right moments in order to reduce the chance of progression of the disease, providing the best outcome to the patient, including the possibility of treatment discontinuation for eligible patients. Although the test is included in the Clinical Protocol and Therapeutic Guidelines (PCDT) of CML, it is not possible to monitor the molecular response within SUS since there is no reimbursement for this test. Objective: Obtain expert recommendations on the importance, financing, and reimbursement of molecular monitoring in SUS. Methods: Six CML experts with different perspectives participated in the panel. The discussion was based in the main publications about the quantitative PCR test in CML monitoring. Results: Experts’ recommendations: 1) Molecular monitoring should be part of the integral treatment of patients with CML to reduce the chances of disease progression and costs to the health system; 2) The government should put into practice what is provided in the PCDT of Chronic Myeloid Leukemia in Brazil: performing the monitoring of the molecular response via quantitative PCR; 3) The government should create a code with adequate nomenclature and reimbursement value in SIGTAP, so that the test is carried out and covered by the public health network, as it is contained in the PCDT of the disease and the existing APAC does not cover the operational costs for its performance; 4) Patients with chronic phase CML should perform a quantitative PCR every 3 months and, after reaching the MMR, should perform the examination every 6 months, as recommended by international guidelines; 5) Patients should be monitored in reference laboratories that are standardized according to the international scale; 6) The laboratories that are within the reference public centers could absorb all the test demand in Brazil, and other centers could be qualified through an ABHH accreditation; 7) Adequate molecular monitoring may allow some patients to stop taking drugs and selffinancing the molecular test for all SUS patients Conclusion: A solution for the molecular test (BCR-ABL1) funding is urgent to ensure the monitoring of CML patients in SUS. The savings that might be generated with patients that stop taking the medication when adequately monitored may finance the test.

The novel Coronavirus (CoVid-19) outbreak is now consider a world pandemic, affecting more than 1,300,000 people worldwide. Cancer patients are in risk for severe disease, including a higher risk of intensive care unit (ICU) admission, need for invasive ventilation or death. Management of patients with lymphoid malignancies can be challenging during the outbreak, due to need of multiple hospital visits and admissions, immunosuppression and need for chemotherapy, radiotherapy and stem cell transplantation. In this article, we will focus on the practical management of patients with lymphoid malignancies during the COVID-19 pandemic, focusing on minimizing the risk for patients.

Abstract Erdheim-Chester Disease is a rare entity, classified as an inflammatory myeloid neoplasm, with an unknown incidence, occurring preferentially in men after 50 years of age. Classically, it has a multisystemic presentation, with the skeletal system being the most frequently affected (90% of the patients), followed by genitourinary involvement in 60% of cases and central nervous system in the pituitary and diabetes insipidus in 25% of the cases. Cardiovascular manifestations are present in more than half of the patients, with aortic infiltration and atrial pseudotumor being the most common forms.