What happens in Leukemias, Lymphomas, and Myelomas?
Tariq I Mughal, John M Goldman, Sabena T Mughal in Understanding Leukemias, Lymphomas, and Myelomas, 2017
The leukemias are a group of disorders characterized by the excessive accumulation of abnormal white cells in the bone marrow and peripheral blood. The lymphomas (a term derived from the Greek word λυμϕos “lymph” meaning the colorless fluid circulating in the special lymphatic vessels of the body; “-oma” is Greek ωμα, a suffix that has no English equivalent) are a heterogeneous group of cancers that originate in lymphoid cells in lymph nodes or other lymphoid tissue. The best characterized of the lymphomas is Hodgkin lymphoma (HL); the remainder constitute a rather motley collection of conditions, ranging from those with a very indolent natural history to those which are very aggressive and, unless treated promptly, can become rapidly fatal. The phrase non-Hodgkin lymphoma (NHL) has been coined to cover this latter group of diseases and, though rather unsatisfactory, no better term has yet been proposed. The name, however, has considerable historical context and we discuss this under the sub-heading of history. It is also intriguing that different leukemias and lymphomas are prone to occur at different ages. Multiple myeloma (MM) (typically referred to as simply “myeloma” or “plasma cell myeloma”) is a cancer which arises from the plasma cells in the bone marrow and is characterized by the production of a single species (monoclonal) of immunoglobulin molecule (a paraprotein; also called M-protein).
The thorax
Spencer W. Beasley, John Hutson, Mark Stringer, Sebastian K. King, Warwick J. Teague in Paediatric Surgical Diagnosis, 2018
The important pathological conditions that affect the mediastinum are cysts and tumours (Table 4.1), although surgical emphysema (pneumomediastinum) and infection (mediastinitis) occur. The site of a tumour or cyst is frequently the key to its nature, although sometimes the final diagnosis is made only at operation. When a superior and/ or anterior mediastinal tumour is present, a pleural effusion is suggestive of lymphoma and examination of the pleural aspirate may enable a diagnosis without the need for direct biopsy of the tumour. Some tumours are asymptomatic and detected incidentally by radiographic examination, while in other cases the size and position of the tumour may lead to compression of vital structures (particularly the airway) and a life-threatening situation. This is particularly true in patients with non-Hodgkin lymphoma.
Second cancers after radiotherapy
Michael C. Joiner, Albert J. van der Kogel in Basic Clinical Radiobiology, 2018
Dores et al. (12) reported results of a large international study on 32,591 Hodgkin lymphoma patients with 2861 patients followed-up for more than 20 years and 1111 patients for more than 25 years. Mean age at treatment was 37 years. Second malignancies developed in 2153 patients (7%) which, compared to the age- and gender-adjusted general population, was an increase of more than a factor of 2. The risk of late-developing solid cancers was particularly increased after radiotherapy, while second leukaemias were mostly related to chemotherapy. The highest excess absolute second cancer risk was for cancers of the lung and breast. While the relative risk of all second cancers decreased with increasing age at diagnosis of Hodgkin lymphoma, the excess absolute risk of second cancers increased with increasing age from 30 cases per 100,000 person-years for less than 21-year-old patients to 107 cases per 100,000 person-years in 51- to 60-year-old patients. This was not seen for second breast cancer, where the risk was highest in patients treated less than 30 years. The authors calculated a 25 years cumulative risk of treatment-induced second cancers of 11.7%, most of which was related to radiotherapy.
Identifying and treating candidates for checkpoint inhibitor therapies in multiple myeloma and lymphoma
Published in Expert Review of Hematology, 2020
Katarina Hradska, Michal Kascak, Roman Hajek, Tomas Jelinek
Classical Hodgkin lymphoma is characterized by the presence of malignant RS cells and the extensive inflammatory microenvironment of immune cells (represented mostly by T cells and macrophages) [65]. RS cells escape immune detection and elimination by hijacking PD-1/PD-L1/2 pathway and with the downregulation of MHC. Upregulation of PD-L1/PD-L2 and JAK2 is driven by almost uniform 9p24.1 genetic alteration (copy gain, amplification) in RS cells and frequent EBV infection in cHL patients [65,68]. Overexpression of PD-1 ligands is more common in the advanced stages of cHL and is associated with inferior PFS [68]. Extensive expression of PD-1 ligands on RS cells, on surrounding macrophages and the simultaneous presence of PD-1 positive tumor-infiltrating lymphocytes (TIL) make cHL an attractive target for checkpoint inhibitor therapy [67].
Paraneoplastic Neurologic Symptoms in a Pediatric Patient with Hodgkin Lymphoma
Published in Cancer Investigation, 2021
Claire C. Baniel, Sarah S. Donaldson, Catherine Aftandilian, Susan M. Hiniker
It is not uncommon for patients with Hodgkin lymphoma (HL) to present with systemic symptoms, including: fever, weight loss, and night sweats, known collectively as “B symptoms” (1). While these are prognostic signs of HL, there are also less common presentations that may be difficult to interpret. Some of these associated symptoms include pruritus, alcohol-associated pain, and liver dysfunction. In addition, there are more rare paraneoplastic symptoms that may also delay timely diagnosis and treatment. Among the paraneoplastic neurological syndromes, those arising in adults have an estimated incidence of less than 0.01%; in children, they are even more rare, with an unknown incidence. Herein, we report a child with a neurological paraneoplastic syndrome comprised of cerebellar ataxia and later, achalasia which led to the diagnosis of EBV + HL.
Clinical prognostic risk analysis and progression factor exploration of primary breast lymphoma
Published in Hematology, 2022
Jili Deng, Lan Mi, Xiaopei Wang, Jun Zhu, Chen Zhang, Yuqin Song
Blood test reports were comprehensively collected to explore better this small group of patients’ adverse prognostic factors. Elevated ESR has previously been suggested as a poor prognostic factor for Hodgkin's lymphoma[24]. In our cohort, other general prognostic risk factors, such as ESR, LDH level, B symptoms, and IPI scores, were equally applicable in PB-DLBCL, showing poor prognostic factors for both OS and PFS (P < 0.05). The stage-adjusted IPI can also identify patients with a survival benefit, even if the disease is confined to an early stage. Although B symptoms in patients with non-Hodgkin lymphoma are frequently neither recorded nor accurate, as mentioned in the Lugano classification[25], they are still crucialin lymphoma patients, especially those with weight loss.
Related Knowledge Centers
- Fever
- Groin
- Weight Loss
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- Lymphadenopathy
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- Night Sweats