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AL amyloidosis is a rare systemic disease characterized by the deposition of amyloid protein in various organs, including the kidneys, heart, peripheral nervous system, digestive tract, skin, and muscles. Peritoneal involvement in AL amyloidosis is exceptionally rare. We present a unique case of AL amyloidosis with concurrent cardiac, cutaneous, and peritoneal manifestations. The patient initially presented with ascites and respiratory symptoms. An etiological workup revealed multiple myeloma as the underlying cause. This case highlights the importance of considering AL amyloidosis in the differential diagnosis of peritoneal ascites, providing valuable insights for radiologists in recognizing atypical presentations of this disease.
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Plasmacytomas are a rare spectrum of plasma cell neoplasms that are single localized tumours, lacking the clinical features of plasma cell myeloma with no radiographical evidence of additional plasma cell tumours. Two clinical variants of plasmacytomas can be distinguished: solitary plasmacytoma of bone and extramedullary (or extraosseous) plasmacytoma. The latter is rare, representing 1% of all plasma cell neoplasms, occurring most frequently in the upper airways. Ovarian localization is exceptional, with only a few cases being reported in the literature. We herein report a case of an ovarian extramedullary plasmacytoma occurring in a 56-year-old woman who consulted for abdominal pain and abdominal mass, while highlighting the main histological and immunohistochemical features of this rare malignancy, along with a thorough review of literature gathering all cases of ovarian plasmacytomas reported to date.
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Neoplasias Ósseas , Mieloma Múltiplo , Plasmocitoma , Feminino , Humanos , Pessoa de Meia-Idade , Plasmocitoma/diagnóstico , Plasmocitoma/patologia , Mieloma Múltiplo/patologia , RadiografiaRESUMO
The diagnosis of chronic lymphoid leukemia (CLL) is essentially based on a blood smear and immunophenotyping by flow cytometry of circulating lymphocytes. Unusual locations of the disease can sometimes be observed. Here we report the case of a patient admitted for the management of pleurisy. The pleural effusion was lymphocytic exudate; histological examination of the pleural biopsy along with immunohistochemistry helped yield the diagnosis of secondary localization of CLL. The patient was transferred to the Internal Medicine department where chemotherapy was introduced.
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Granulomatous mastitis is an inflammatory disease that often affects women with a history of breastfeeding. The pathogenesis is still unclear and several factors have been incriminated, such as trauma, metabolic and hormonal disorders, infections, and autoimmunity. This poses a diagnostic issue, given that there are several different diagnoses, particularly carcinomatous mastitis. We report the case of a 32-year-old woman, with a history of breastfeeding, who presented with inflammatory left breast. The physical examination has objectified a 10/10 cm painless mass and a 3 cm homolateral axillary lymphadenopathy. A sonomammography revealed inflammatory left breast infiltration with multiple collections associated with homolateral axillary lymphadenopathies. A Trucut biopsy was performed, revealing granulomatous mastitis without signs of malignancy. Interferon-gamma measurement and Koch Bacillus (BK) search by polymerase chain reaction (PCR) in the breast collection were all negative. The patient was put on non-specific antibiotics with no response and clinical worsening; therefore, we were obliged to start bacillary treatment. The evolution was marked by a total drought and the disappearance of inflammatory signs within a few weeks. Mammary tuberculosis poses a diagnostic issue given the difficulty to identify the bacteria in the samples. This is why tuberculosis should never be excluded despite negative results, especially in endemic countries.
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Focal segmental glomerulosclerosis is a severe renal disease with a complex and unclear pathophysiology. Nephrotic syndrome is the clinical presentation of this renal disease. The recurrence of the disease after renal transplantation and the remission obtained after immune-adsorption treatment illustrate the implication of a circulating factor that requires characterization. Granulomatous inflammation is a tissue reaction caused by several conditions, including neoplastic diseases. In the literature, focal segmental glomerulosclerosis and granulomatous inflammation have both been associated with lymphoma. We report the case of a 56-year-old woman who initially developed focal and segmental glomerulosclerosis. After one year, the granulomatous inflammation was treated as tuberculosis infection and then as sarcoidosis. Finally, after another year, non-specified peripheral T-cell lymphoma was diagnosed.
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The incidence of lymphoma is constantly increasing worldwide. The reasons for this increase are unclear and likely multiple. B cell lymphomas represent the majority of non-Hodgkin lymphomas. Primary orbital localization remains a rare entity to think about in order to avoid missing a therapeutic emergency. In this article, we report the case of a 52-year-old man who has been treated for five years for an inflammatory orbitopathy with steroids, but the worsening of the clinical condition and the installation of exophthalmia led to push investigations and the outcome was a primary orbital lymphoma marginal zone type. The patient was treated by systemic chemotherapy with immunotherapy (RCHOP protocol) with a very good evolution and complete disappearance of the lesion after chemotherapy.
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Skin involvement in Hodgkin's lymphoma (HL) is rare. The diagnosis can be difficult, mainly due to the wide range of cutaneous lesions that can be observed, but also due to the differential diagnosis, even after the immunohistochemical staining. We present the case of a 30-year-old man who presented with a painful cutaneous nodular lesion; biopsy and immunohistochemical stains were consistent with classic HL. The patient was treated with adriamycin, bleomycin, vinblastin, and dacarbazine (ABVD) with complete remission.
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Retroperitoneal fibrosis is a rare disease manifesting as chronic soft tissue fibrosis in the retroperitoneum, with potential anatomic and/or functional compromise of adjacent organs. It can be primary (idiopathic) or secondary to other conditions such as cancers, radiotherapy, surgery, traumatisms, infections, autoimmune disorders, or drugs. We report herein a 54-year-old patient with symptomatic retroperitoneal fibrosis leading to bilateral hydronephrosis and renal failure, in whom, after a complex diagnostic workup and protracted clinical course, a follicular lymphoma in the retroperitoneal was identified. The patient was treated with rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy protocol, with a resolution of hydronephrosis and lower back pain. We include a thorough literature review on etiopathogenesis, diagnosis, therapy, and prognosis of retroperitoneal fibrosis. A meticulous search for malignancy is necessary for this rare condition that, if positive, may have significant therapeutic and prognostic implications.
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Raynaud's phenomenon (RP) is a frequent syndrome and often indicative of connectivitis or hemopathy. The association with solid cancers is exceptional. We report the observation of a patient hospitalized for severe RP whose etiological assessment revealed the existence of colorectal cancer. We discuss, through this clinical case, the potential physiopathological links and underline the importance of looking for underlying cancer in the face of severe, refractory to treatment, or atypical RP.
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Primary diffuse large B cell lymphoma of the conjunctiva is a rare disease. In this article, we report the case of a 40-year-old man who had previously been treated with chemotherapy and radiotherapy for undifferentiated carcinoma of nasopharyngeal type (UCNT) and who subsequently developed conjunctival lymphoma. We underline through this observation the importance of thinking about a secondary cancer post-radio-chemotherapy even when the clinical presentation is atypical.
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Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder due to autoantibodies directed against nuclear and cytoplasmic antigens that may affect several different organs. The association of SLE and acute myeloid leukemia (AML) is rare, the incidence of this combination is not known, but there are few case reports in the literature. We report here the case of a 62-year-old woman, admitted for aetiological diagnosis of weight loss and severe anemic syndrome. The blood count has objectified a pancytopenia with lymphopenia. A thorough assessment was carried out following which a diagnosis of AML associated to SLE was retained. The patient received a low dose of cytarabine due to comorbidities and poor performance status associated with steroids and she died three months after diagnosis with a septic shock.
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BACKGROUND: Biobanks are highly organized infrastructures that allow the storage of human biological specimens associated with donors' personal and clinical data. These infrastructures play a key role in the development of translational medical research. In this context, we launched, in November 2015, the first biobank in Morocco (BRO Biobank) in order to promote biomedical research and provide opportunities to include Moroccan and North African ethnic groups in international biomedical studies. Here, we present the setup and the sample characteristics of BRO Biobank. METHODS: Patients were recruited at several departments of two major health-care centers in the city of Oujda. Healthy donors were enrolled during blood donation campaigns all over Eastern Morocco. From each participant, personal, clinical, and biomedical data were collected, and several biospecimens were stored. Standard operating procedures have been established in accordance with international guidelines on human biobanks. RESULTS: Between November 2015 and July 2020, 2446 participants were recruited into the BRO Biobank, of whom 2013 were healthy donors, and 433 were patients. For healthy donors, the median age was 35 years with a range between 18 and 65 years and the consanguinity rate was 28.96%. For patients, the median age was 11 years with a range between 1 day and 83 years. Among these patients, 55% had rare diseases (hemoglobinopathies, intellectual disabilities, disorders of sex differentiation, myopathies, etc.), 13% had lung cancer, 4% suffered from hematological neoplasms, 3% were from the kidney transplantation project, and 25% had unknown diagnoses. The BRO Biobank has collected 5092 biospecimens, including blood, white blood cells, plasma, serum, urine, frozen tissue, FFPE tissue, and nucleic acids. A sample quality control has been implemented and suggested that samples of the BRO Biobank are of high quality and therefore suitable for high-throughput nucleic acid analysis. CONCLUSIONS: The BRO Biobank is the largest sample collection in Morocco, and it is ready to provide samples to national and international research projects. Therefore, the BRO Biobank is a valuable resource for advancing translational medical research.
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Bancos de Espécimes Biológicos/ética , Bancos de Espécimes Biológicos/normas , Pesquisa Biomédica/normas , Manejo de Espécimes/ética , Manejo de Espécimes/normas , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Doadores de Sangue/ética , Criança , Pré-Escolar , Consanguinidade , Etnicidade , Feminino , Geografia , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Marrocos , Controle de Qualidade , Pesquisa Translacional Biomédica , Adulto JovemRESUMO
The diagnostic management of systemic granulomatosis is a difficult clinical exercise. The most frequent etiologies are tuberculosis and sarcoidosis. However, it is important to search as well for the other causes of granulomas, especially infections and malignancies, the prognosis of which can be poor without adequate treatment. A 67-year-old immunocompetent patient presented with granulomatous adenitis without caseous necrosis. The etiological evaluation had revealed neurological, pulmonary and lymph node systemic cryptococcosis. Conventional antifungal therapy with the triple combination Amphotericin B - Flucytosine - Fluconazole has not been effective, indicating administration of voriconazole. The evolution was rapidly favorable with apyrexia after 48 hours, disappearance of clinical symptoms, normalization of biological parameters of cerebrospinal fluid (CSF) and major improvement of radiological abnormalities. This clinical case is original by the disseminated involvement, the patient's non-immunocompromised status and the primary resistance to amphotericin B. Our findings underline the importance of carrying out an exhaustive evaluation, reflecting on cryptococcosis in any systemic granulomatosis and knowing the various therapeutic alternatives, in particular, voriconazole if primary response to amphotericin B has not been obtained.