Plasma Cell Neoplasms
Tariq I. Mughal in Precision Haematological Cancer Medicine, 2018
The 2016 World Health Organization classification defines several distinct groups under the heading of mature B-cell neoplasms: plasma cell myeloma and solitary plasmacytoma of bone and extraosseous plasmacytoma. This chapter addresses some of the biological advances, and how they have impacted the diagnosis, staging, prognosis and the management of patients with the disorders. Myeloma cells are phenotypically like older plasma cells with a low rate of proliferation and a strong dependency on the bone marrow microenvironment for growth and survival, which they retain until the advanced stages. Patients with a solitary bone-related plasmacytoma have a localized disease and conventional treatment involves localized radiotherapy. Patients with a solitary extramedullary plasmacytoma appear to have a truly localized disease with very few patients relapsing and thus it rarely progresses to myeloma. Prognostic classification incorporates serum free light chain, cardiac biomarkers, renal function and plasma cells.
Muscle, Bone, and Skin Disorders
Victor A. Bernstam in Pocket Guide to GENE LEVEL DIAGNOSTICS in Clinical Practice, 2019
The tissue-specific expression of the dystrophin gene in muscle tissue and brain is explained by the fact that the brain promoter is active only in neurons, whereas the muscle promoter operates in skeletal, cardiac, and smooth muscle as well as in glial and neuronal cells. Moreover, selective reduction in dystrophin expression in the brain or muscle may be due to a deletion of either brain-specific or muscle-specific promoter. Osteosarcoma is the most common malignant tumor of bone, excluding plasma cell myelomas. The diversity of skin pathologies and lack of concentrated effort lead only to fragmentary observations on the biology of keratinocytes affected by some skin disorders. The triumphant discoveries of the gene and its product, dystrophin, responsible for Duchenne muscular dystrophy, and a related milder, allelic form, Becker muscular dystrophy, allowed the development of molecular tools for unequivocal differentiation of these muscular disorders from a number of clinically similar diseases.
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 are a heterogeneous group of cancers that originate in lymphoid cells in lymph nodes or other lymphoid tissue. The common feature of these tumors was that the component cells all seemed to be derived from the lymphatic system. Lymphomas, in particular Non-Hodgkin lymphoma, in contrast to leukemia are considerably more common worldwide. Multiple myeloma is a cancer which arises from the plasma cells in the bone marrow and is characterized by the production of a single species of immunoglobulin molecule. The different subtypes of myeloma were not recognized until the 1930s, when electrophoresis was discovered. In tandem with the leukemias and lymphomas, much of the molecular understanding of myeloma has been achieved. Amongst the leukemias, perhaps up to 5% may arise from inherited abnormal genes.
Disseminated plasma cell myeloma presenting as massive pleural effusion
Published in European Clinical Respiratory Journal, 2015
Kanahasubramanian Anand Babu, Lakshmikanthan Sundararajan, Pandurangan Prabu, Ashok Parameswaran
Plasma cell myeloma (PCM) is a hematologic malignancy of plasma cell origin and usually associated with the presence of lytic bone lesions. Pleural effusions are rarely associated with PCM and most often signify a concurrent disease process. Malignant myelomatous pleural effusions are even more unusual and carry a poor prognosis. We report a unique case of unsuspected PCM with thoracic involvement in the form of massive left side pleural effusion. Pleural fluid cytology revealed numerous atypical plasma cells. Subsequently on further workup, urine Bence Jones protein was positive. Bone marrow aspiration and biopsy and computed tomography of the chest and abdomen revealed features consistent with multiple myeloma.
Plasma cell leukemia: update on biology and therapy
Published in Leukemia & Lymphoma, 2017
Roberto Mina, Mattia D’Agostino, Chiara Cerrato, Francesca Gay, Antonio Palumbo
Plasma cell leukemia (PCL) is a rare, but very aggressive, plasma cell dyscrasia, representing a distinct clinicopathological entity as compared to multiple myeloma (MM), with peculiar biological and clinical features. A hundred times rarer than MM, the disease course is characterized by short remissions and poor survival. PCL is defined by an increased percentage (>20%) and absolute number (>2 × 109/l) of plasma cells in the peripheral blood. PCL is defined as ‘primary’ when peripheral plasmacytosis is detected at diagnosis, ‘secondary’ when leukemization occurs in a patient with preexisting MM. Novel agents have revolutionized the outcomes of MM patients and have been introduced also for the treatment of PCL. Here, we provide an update on biology and treatment options for PCL.
Plasma cell cheilitis, successfully treated with topical 0.03% tacrolimus ointment
Published in Journal of Dermatological Treatment, 2010
Seon Pil Jin, Kwang Hyun Cho, Chang Hun Huh
Plasma cell cheilitis is a rare, idiopathic mucosal condition. The treatment of plasma cell cheilitis is often disappointing. It is often resistant to various topical treatments. We present a 65-year-old woman who had a painful, eroded area on her lower lip, which responded poorly to various topical treatments. A biopsy revealed a band-like infiltration composed mainly of plasma cells in the dermis. She was diagnosed as having plasma cell cheilitis, and was successfully treated with 0.03% topical tacrolimus ointment.
Related Knowledge Centers
- Lymphocytes
- White Blood Cell
- Lymphatic System
- Bone Marrow
- Immunoglobulin
- B-Lymphocytes
- Blood Plasma