Plasmacytoma

Plasmacytoma is a malignant tumor of plasma cells. It usually affects bone marrow, where it causes bone damage such as pathological fractures. If there are present more foci, we call it multiple myeloma (Kahler's disease). Myeloma cells produce paraprotein. Although, rarely non-secretory myelomas may occure, in which no paraprotein can be detected in serum.

Macroscopy
Myeloma takes the form of dark red osteolytic deposits in the bone marrow (calvaria, vertebrae, pelvis, humeral shaft, femur.

Complications

 * Pathological fractures (there is a great risk of spinal cord lesions in vertebrae).
 * AL amyloidosis.
 * Myocardium - heart rhythm disorders, heart failure.
 * Large intestine - malabsorption.
 * Kidneys - renal failure (proteinuria, periorbital edema, uremia).
 * Liver, blood vessels, lymph nodes.
 * Tongue - macroglossia.
 * Myeloma kidney - obstruction of tubules by protein chains - Bence Jones protein.

Life-threatening issues

 * Failure of an organ affected by amyloidosis,
 * bronchopneumonia - develops after vertebral fracture and spinal cord injury,
 * bone marrow infection due to generalization of the tumor (same as in leukemia).

Clinical description




Epidemiology
The disease is more prevalent in men than in women (1.5: 1). The incidence is rising from the age of 50, the median age is 70 years. The incidence in the Czech Republic is 3-4: 100,000 inhabitants / year. The etiology of the disease is unknown. It occurs hardly ever in childhood and in young adults.

Clinical picture


The initial period may be asymptomatic (in indolent lymphomas), but pain in the back and ribs is present in over 70% of patients. Pain affects the physical activity.

The clinical picture consists of a set of symptoms hidden under the abbreviation of CRAB:
 * Calcium – hypercalcemia, which is associated with bone degeneration. Hypercalcaemia also causes depression, letargy and a tiredness.
 * Renal failure – renal failure due to tubulopathy, an uncommon symptom of myeloma,
 * Anemia – normocytic normochromic anemia is the most common symptom, it is associated with weight loss, fatigue, shortness of breath and paleness
 * Bone – bone disorder - very common, tumor cells in the bone marrow produce osteoclasts activating factors, which leads to osteolysis of the bones and the formation of pathological fractures, which occur in up to 70% of patients.

Susceptibility to infections, manifestations of hyperviscous syndrome (headache, vision disorders) and palpable infiltrates over osteolytic deposits (especially on the skull) also contribute to the clinical picture.

Diagnostic methods

 * X-RAY - reveals pathological lesions (with often affected skull and spine, but myeloma can basically occur in any bone), as well as CT, MRI, PET,
 * determination of paraprotein in blood – electrophoresis of plasma proteins (see pictures), concentration of paraprotein in serum is usually> 30 g / l,
 * The serum immunoglobulin-free light chain assay (FLC) – the most sensitive method, it also detects non-secretory myeloma, in which the paraprotein determination would be negative, the normal ratio of kappa-lambda light chains is 2:1, in the case of myeloma it could for example be, 350: 1,
 * determination of Bence Jones protein in urine,
 * bone marrow examination – the number of plasma cells usually exceeds 10% of all nuclear cells in the bone marrow, as well as immunohistochemical and cytogenetic examination (occurrence of deletions and trisomies has its prognostic significance),
 * cytogenetic examination - numeric and structural changes in chromosomes, important for prognosis,
 * laboratory examination,
 * normocytic normochromic anemia, mild leukopenia and thrombocytopenia
 * increased sedimentation,
 * hypercalcemia,
 * renal functions - increased urea and creatinine,
 * examination of LD, thymidine kinase and beta2-microglobulin, hyperuricemia - their increased values ​​have a negative prognosis.

Clinical stages
The Salmon-Durie classification of MM :
 * Stage I – clinical stage I - paraprotein concentration of IgG <50 g / l, or IgA <30 g / l, proteinuria <4 g / 24 hours, no osteolytic changes without hypercalcemia,
 * StageII – values ​​are between stages I and III,
 * Stage III – concentration of IgG paraprotein > 70 g / l, or IgA> 50 g / l, proteinuria >12 g / 24 hours, multiple bone deposits, hypercalcemia > 2.75 mmol / l, concentration of hemoglobin <85 g / l,
 * subclassifications either A or B according to the value of serum creatinine (renal impairment),
 * A subclassification – serum creatinine ≤ 177 μmol/l,
 * B subclassification – serum creatinine > 177 μmol/l.

Clinical variants
Asymptomatic myeloma, symptomatic myeloma,
 * smoldering myeloma,
 * indolent myeloma,
 * stage I multiple myeloma,
 * stage II and III multiple myeloma,
 * solitary plasmacytoma.

Therapy

 * If myeloma happens to be asymptomatic, it does not have to be treated, only monitored,
 * if symptomatic, it is treated as following:
 * younger patients are indicated for autologous hematopoietic stem cell transplantation (aHSCT),
 * elderly patients are indicated for chemotherapy,
 * chemotherapy – cyclophosphamide and dexamethasone (or thalidomide) are standardly used, bortezomib has been registered in the Czech Republic in 2009 for primary treatments,
 * radiotherapy – used for painful bone deposits,
 * palliative care – in patients with severe comorbidities, melphalan and prednisone, or radiotherapy.

Related articles

 * Amyloidosis