The lymphoreticular system and bone marrow
C. Simon Herrington in Muir's Textbook of Pathology, 2020
Thymoma is a primary epithelial tumour of the thymus that behaves like a low-grade carcinoma, invading surrounding structures but seldom metastasizing (<10% of cases). The lesion consists of epithelial cells, often interspersed with so many thymic lymphocytes that it can be misdiagnosed as a lymphoma. Although often found incidentally, thymoma may be associated with myasthenia gravis, pure red cell aplasia, hypogammaglobulinaemia, and autoimmune diseases such as polymyositis. Several forms of lymphoma may affect the thymus, most notably classical Hodgkin lymphoma and mediastinal large B-cell lymphoma, both typically in young females. T-lymphoblastic leukaemia, which usually occurs in childhood, may present with a large anterior mediastinal mass. Germ cell tumours, similar to those found in the gonads, can arise at this site.
The heart, lungs and pleura
Kevin G Burnand, John Black, Steven A Corbett, William EG Thomas, Norman L Browse in Browse’s Introduction to the Symptoms & Signs of Surgical Disease, 2014
Masses arising in the mediastinum are inaccessible to clinical examination. Modes of presentation are: no symptoms, with an incidental finding on imaging studies such as a chest X-ray or CT scan;chest pain, normally central; paravertebral masses may produce pain in the affected dermatome;shortness of breath as a consequence of airway compression or invasion by large mediastinal masses or lymphadenopathy;cough;dysphagia from compression of the oesophagus;fever or night sweats in mediastinal lymphadenopathy caused by lymphoma or tuberculosis;general malaise;muscle weakness secondary to myasthenia gravis associated with a thymoma.
Mediastinal tumours
Anju Sahdev, Sarah J. Vinnicombe in Husband & Reznek's Imaging in Oncology, 2020
Thymomas have a wide spectrum of histological diversity and are classified based on cell type predominance as lymphocytic, epithelial, or spindle cell variants. Invasive thymomas are indistinguishable from benign lesions histologically. There is, however, strong association between histological subtype and invasiveness, as well as prognosis (40–42). The World Health Organization (40,41) developed a classification system to group thymomas based on cytologic differences, which may be helpful in determining treatment regimens and predicting prognosis (Table 5.3). In contrast to histological classification, the stage of thymoma has clinical implications and it is a useful tool for management decisions. The Masaoka-Koga staging system is the most commonly used and describes thymomas in terms of local extension to stratify 5-year survival rates (Table 5.4). The Masaoka staging, together with the completeness of surgical resection, most strongly correlates with prognosis of thymomas (42–44).
Respiratory insufficiency from myasthenia gravis and polymyositis due to malignant thymoma triggering Takotsubo syndrome
Published in International Journal of Neuroscience, 2018
Josef Finsterer, Claudia Stöllberger, Chen-Yu Ho
Myasthenic crises have been repeatedly reported to trigger TSS (Table 1). The current patient, however, is unique since MG was associated with malignant thymoma and since the response to cholinergic drugs and plasmapheresis was poor. Including the present case, 15 patients have been reported so far in whom TTS was triggered by complications of MG (Table 1) [4–17]. Thirteen patients were female and two were males. Age at onset of MG ranged from 40 to 83 years. In four patients, a thymoma was diagnosed. In three patients, it was not reported whether there was a thymoma or not. The type of TTS was reported in 13 patients, 12 patients presented with the classical type and a single patient had the global type. The presumed trigger of TTS was a myasthenic crisis in 14 patients and unknown in one patient (Table 1). Only seven patients received treatment for TTS. The outcome of TTS was reported in 13 patients. Among these, full recovery was achieved in 10 patients (76.9%). In 3/13 patients (23%), the outcome was fatal.
Eculizumab for the treatment of myasthenia gravis
Published in Expert Opinion on Biological Therapy, 2020
Renato Mantegazza, Paola Cavalcante
A consensus-based guidance for MG management has been developed by a panel of 15 international experts, convened by a Task Force of the MGFA, to be a guide for clinicians worldwide [14,15]. A stepwise treatment approach is recommended for gMG, beginning with cholinesterase inhibitors, that prolong acetylcholine availability at the NMJ (first-line treatment), followed by immunosuppressive therapy (IST) with corticosteroids, used chronically in most patients [14–16]. If corticosteroids are not effective or exhibit contraindications, patients can be treated with other IS agents, alone or combined with steroids (second-line treatment). Immune globulin (IVIg) and plasmapheresis (PE) are recommended for MG exacerbation or crises to obtain rapid improvement [14–16]. Finally, thymectomy, mandatory for thymoma patients, is able to improve the disease course in non-thymomatous patients [17,18].
Successful treatment of acquired amegakaryocytic thrombocytopenia with eltrombopag and immunosuppressant
Published in Platelets, 2022
Hong Tian, Danqing Kong, Yun Li, Chengyuan Gu, Ziqiang Yu, Zhaoyue Wang, Depei Wu, Jie Yin
A 15-year-old male was hospitalized for severe thrombocytopenia (1 × 109/L) in April 2020. The blood smear showed no blasts or schistocytes. Bone marrow aspiration and biopsy were performed, which showed normal hematopoiesis with an absence of megakaryocytes. The flow cytometry of bone marrow and karyotype did not show any abnormalities. No evidence of TCR/IgH rearrangement was reported. Thyroid function was normal, while thyroid peroxidase antibody and anti-thyroglobulin antibody were positive. Abdominal ultrasonography showed a normal liver and spleen size. CMV, EBV, hepatitis C, and HIV were all negative. The monoclonal antibody-specific immobilization of platelet antigen (MAIPA) test was within the normal range. Antinuclear antibody (ANA) testing revealed that ANA (1:100), anti-mitochondrial antibody (AMA)-M2, anti-Ro-52 antibody, and anti-PM/Scl-100 antibody were positive. No evidence of thymoma was seen on computerized tomography (CT). Based on all the aforementioned data, a diagnosis of AAMT was made.