BACKGROUND: For a patient presenting with fever, multiple lymphadenopathy and splenomegaly, pathogen infection should be preferentially considered, followed by lymphoid malignancies. When traditional laboratory and pathological detection cannot find the pathogenic microorganism, metagenomic sequencing (MGS) which targets the person\'s genome for exceptional genetic disorders may detect a rare pathogen.
METHODS: Here, we introduced the diagnostic clue of a case of multicentric Castleman disease (MCD) with hemophagocytic syndrome which was elicited from the detection of human herpesvirus-8 in the blood of a HIV-1 infected person by MGS technology during pathogen inspection. This case highlights the need to increase the awareness of MCD among clinicians and pathologists.
CONCLUSIONS: MGS technology may play a pivotal role in providing diagnostic clues during pathogen inspection, especially when pathogens are not detectable by conventional methods.

BACKGROUND: Human herpes virus-8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder driven by proinflammatory cytokines, which is still poorly understood. Pulmonary parenchyma lesion is a rare condition in iMCD, which mainly manifests as lymphocytic interstitial pneumonia and is an indicator of severe iMCD. Cutaneous lesion is also very rare and mainly occurs in Asians. There have been few reports of iMCD patients with both skin and lung parenchyma involvement.
METHODS: We present a Chinese man who complained about a 3-year history of intermittent dry cough and a 2-year history of diffuse reddish-brown maculopapules. Laboratory examination revealed polyclonal hypergammaglobulinemia and hypercytokinemia including interleukin 6. Chest computed tomography revealed small patchy shadows with ground-glass nodules scattered in two lobes and mediastinal lymphadenopathy. The pathological result of the lymph node was consistent with the plasma cell type of Castleman disease. As serum human immunodeficiency virus test and HHV-8 staining of the lymph node were negative, the patient was finally diagnosed with HHV-8 negative iMCD. He was treated with tocilizumab at an intravenous (i.v.) dose of 8 mg/kg every 2 wk combined with methylprednisolone at an i.v. dose of 80 mg/d initially with gradual dose tapering. Partial remission was achieved 9 mo later.
CONCLUSIONS: iMCD with lung parenchyma and skin involvement is a rare condition that requires clinicians\' attention and awareness for early diagnosis.

暂无摘要。

CD20 expression is absent in a variety of diffuse large B cell lymphomas (DLBCLs), including plasmablastic lymphoma, primary effusion lymphoma, anaplastic lymphoma, kinase-positive DLBCL, and large B cell lymphoma arising in human herpesvirus 8-associated multicentric Castleman disease. These rare and heterogeneous tumors are characterized by the presence of proliferating immunoblasts with similar transcriptional profiles as those of plasma cells and are typically associated with highly aggressive pathologies, with high levels of chemotherapy resistance and low survival rates; thus, they pose significant diagnostic and treatment challenges. We conducted a systematic literature review of the limited existing clinical data to summarize the current knowledge regarding the biological basis, diagnostic limits, and potential therapeutic targets of distinct variants of CD20-negative DLBCL. This review will hopefully increase the awareness of these rare disorders among clinicians and pathologists and prompt basic and clinical research.

A 39-year-old female presented to The First Affiliated Hospital of Jishou University (Jishou, Hunan) with a fever of unknown origin and progressive abdominal distension. Physical examination revealed generalized lymphadenopathy, multiple non-tender cutaneous nodules, hepatomegaly, splenomegaly and abdominal edema. An axillary lymph node biopsy indicated hyaline vascular type Castleman disease, and color Doppler and computed tomography scans suggested Budd-Chiari syndrome (BCS). Based on the abdominal distension and impairments of the liver and kidneys, an inferior vena cavography and balloon dilatation were performed, confirming the diagnosis of BCS and leading to symptomatic improvement. The patient commenced a combination chemotherapy regimen of cyclophosphamide (0.4 g; days 1-3), vindesine (4 mg; day 1) and prednisolone (100 mg; days 1-5), with no melioration of symptoms. Theprubicin was added to suppress the aggravation of the disease on day six of the chemotherapy cycle. The patient exhibited symptomatic remission for one week, however, she subsequently succumbed to intracranial hemorrhage and infections of the lung and intestine due to long-term myelosuppression following chemotherapy. To the best of our knowledge, this is the first report of BCS in a patient with multicentric Castleman disease without human immunodeficiency virus infection.