In accordance with ICD-10, paraproteinemic hemoblastosis is classified as a class 2 neoplasm (C00-D48), part C81-C96. This includes malignant tumors of hematopoietic, lymphoid and related tissues.
They belong to the group of tumor diseases of the circulatory system, the main symptom of which is the secretion of paraproteins or/and their fragments. In different patients, paraproteins can reach significant concentrations in blood serum and belong to different classes. The source of tumor growth is B-lymphocytes.
Paraproteinemic hemoblastoses are common throughout the world. With the age of the patient, their frequency only increases.
Classification of paraproteinemic hemoblastoses
Forms of hemoblastoses are isolated depending on what immunoglobulins they secrete, and on the morphological characteristics of the tumor substrate:
- lg-secreting lymphomas;
- multiple myeloma;
- heavy chain diseases;
- acute plasmablastic leukemia;
- macroglobulinemiaWaldenström;
- solitary plasmacytoma.
Common clinical signs
What are the signs of paraproteinemic hemoblastoses?
The clinical picture is characterized by the presence of a tumor that produces a paraprotein, and secondary humoral immunodeficiency, which develops in all patients when there is an increase in tumor mass. According to the nature of the course of the disease, the stage is chronic (expanded) and acute (terminal).
Paraproteinemia causes common manifestations for pg:
- Peripheral neuropathy.
- Increased blood viscosity.
- Microcirculatory disorders.
- Hemorrhagic syndrome.
- Kidney damage.
- Cryoglobulinemia type 1-2, amyloidosis.
This is the most common classification of paraproteinemic hemoblastoses. Let's consider each type in more detail.
Multiple myeloma
Multiple myeloma is considered the most common PG with unclear reasons for its development. The morphological picture is represented by plasma cells of a certain degree of maturity, often with features of atypism. The expanded stage is characterized by the localization of the tumor in the bone marrow, sometimes in the lymph nodes, spleen, liver.
The nature of the distribution of lesions in the bone marrow allows us to distinguish several forms of multiple myeloma: diffuse-focal, diffuse and multiple-focal.
Bone around the tumor foci is destroyed, because the multiplemyeloma stimulates osteoclast activity. The osteolytic process in various forms has features. For example, the diffuse-focal form is characterized by osteoporosis, which causes the appearance of foci of osteolysis; diffuse - observation of osteoporosis; multiple-focal - possible individual foci are osteolytic. The expanded stage of the tumor usually does not affect the destruction of the cortical layer of the bone. She thins it and lifts it, forming swelling on the skull, sternum, and ribs. The terminal stage of the tumor is characterized by the formation of through defects and germination in the soft tissues around.
Types of myeloma
The class of secreted immunoglobulins affects the isolation of several types of multiple myeloma: A-, D-, G-, E-myeloma, Bence-Jones type l or c, non-secreting.
Myeloma is divided into 3 stages based on the analysis of blood creatinine, hemoglobin, paraprotein levels in urine and blood serum, bone radiographs.
- Stage 1 - the tumor weighs less than 600 g/m2.
- 2 stage - 600 to 1200 g/m2.
- 3 stage - over 1200 g/m2.
The absence or presence of kidney failure affects the assignment of the symbol stage A or B. What are the symptoms of this form of paraproteinemic hemoblastosis?
Tumor has a varied clinical picture. The appearance of the first signs usually occurs in the third stage (weakness, fatigue, pain). Consequenceosteodestructive process is the development of pain. The most common pain in the defeat of the sacrum and spine. Quite often, the ribs, sections of the shoulder and femur bones, affected by the tumor, hurt. Extradural localization of foci is characterized by the fact that compression of the spinal cord follows a pronounced pain syndrome.
Diagnosis is established on the basis of data obtained after sternal puncture and the detection of monoclonal immunoglobulins in the urine and / or blood serum. X-ray examination of the skeleton is of additional importance. If the patient has a multiple-focal form, then a puncture of the sternum may not reveal a tumor.
After the diagnosis of paraproteinemic hemoblastoses (a photo of the tumor can be found in the article) is established, a fluoroscopic examination of the skeleton is carried out before the start of treatment, the function of the liver and kidneys is checked. The use of excretory urography and other aggressive methods of examining the kidneys is not allowed, as they can provoke the development of their irreversible acute failure.
Myeloma therapies
Typically, treatment begins in a hematology hospital, and then it can be carried out on an outpatient basis.
In cases where individual foci of osteolysis create a threat of a pathological fracture, mainly of the supporting parts of the skeleton, if there are separate large tumor nodes of any localization, the first symptoms of spinal compression, the postoperative period afterdecompressive laminectomy, then radiation therapy is recommended.
Solitary plasmacytoma
What other paraproteinemic hemoblastoses are?
Solitary plasmacytoma is a local tumor. The clinical picture depends on the size and location. Most often, solitary plasmacytomas are early-stage multiple myeloma. Bone solitary plasmacytoma is prone to generalization, detected as multiple myeloma 1-25 years after radical therapy has been performed.
Extraosseous solitary plasmacytoma can be localized in any organ, but mainly in the upper respiratory tract and nasopharynx. 40-50% of patients suffer from bone metastases.
The diagnosis is based on the data of morphological examinations of biopsy or puncture material. Multiple myeloma must be ruled out before starting treatment. Radical surgery and/or radiation therapy is used for treatment, with the help of which 50% of patients are completely cured. Patients with solitary plasmacytoma are under observation for life due to the fact that generalization of the process is possible.
This is not all types of paraproteinemic hemoblastoses.
Waldenström macroglobulinemia
Waldenström's macroglobulinemia is a chronic, in most cases, subleukemic or aleukemic lymphocytic leukemia. Localization of lgM-secreting tumor occurs in the bone marrow. Characterizes its lymphocytic composition of cells with admixtureplasma. In addition to monoclonal lgM, Bence-Jones protein is secreted by tumor cells in approximately 60% of patients. Waldenström's macroglobulinemia is much less common than multiple myeloma.
The most common clinical manifestations include bleeding and hyperviscosity syndrome. You can also observe peripheral neuropathy, secondary immunodeficiency, amyloidosis, kidney damage. Rarely, renal failure develops. The advanced stage is characterized by enlargement of the liver, spleen and/or lymph nodes, weight loss in 50% of patients. Anemia develops late, leukocytes may be normal, the leukocyte formula is unchanged, lymphocytosis with moderate leukocytosis is quite common, and some neutropenia is possible. A sharply increased ESR is common.
Diagnosis is made on the basis of immunochemical determination of monoclonal lgM in the blood, trepanobiopsy data or sternal puncture, urine and serum protein electrophoresis. Therapy is carried out in a hematological hospital. What else is paraproteinemic hemoblastosis?
Heavy chain diseases
Heavy chain diseases are very diverse in clinical and morphological characteristics. Their features include the presence of an abnormal protein in the urine and / or blood serum. A-, g-, m-heavy chain diseases are distinguished.
A-disease is the most common, affecting mainly children and young people under the age of 30. The disease is common in the Middle and Near East, in the countries of the Mediterranean basin. Has twoforms of leakage: pulmonary and abdominal (pulmonary is extremely rare). The clinical picture is determined by the syndrome of impaired absorption, amenorrhea, chronic diarrhea, baldness, steatorrhea, hypokalemia, exhaustion, hypocalcemia, edema. Possible abdominal pain and fever.
Description of g heavy chain disease (Franklin's disease) occurs in only a few dozen patients. Morphological data and the clinical picture are diverse, unspecific. The most commonly noted proteinuria, relative neutropenia, thrombocytopenia, progressive anemia, abnormal fever, Waldeyer ring lesion with swelling of the tongue, soft palate and erythema, enlarged liver, lymph nodes and spleen. The course of the disease is usually rapidly progressive and severe. Within a few months, death sets in.
Heavy chain disease m is the rarest form. As a rule, older people are affected by the disease. The disease manifests itself in the form of subleukemic or aleukemic lymphocytic leukemia, usually the lymph nodes are not enlarged, but the spleen and / or liver are enlarged. Some patients have amyloidosis and osteodestruction. And in the bone marrow of almost every patient, lymphocytic infiltration is detected. Many lymphocytes are vacuolated, there may also be an admixture of plasma and lymphoblasts, plasma cells.
It is extremely difficult to make a diagnosis based on the clinical picture. Its establishment occurs through immunochemical methods that detect heavy immunoglobulin a-, g-or m-chains. Treatment of paraproteinemic hemoblastoses of this type takes place in a hematological hospital.
lg-secreting lymphoma
lg-secreting lymphoma - a tumor that has predominantly extra-medullary localization, often they are highly differentiated (lymphoplasmocytic, lymphocytic), rare-blast, that is, sarcomas. The difference from other lymphomas is the secretion of monoclonal immunoglobulins, often of the M-class, a little less of the G-class and extremely rarely of the A, including Bence-Jones protein. It is diagnosed and treated according to the same principles as lymphomas that do not secrete immunoglobulins. If there are symptoms that are caused by paraproteinemia, then the methods of prevention and treatment are the same as for Waldenström's macroglobulinemia and multiple myelomas.
Next, let's look at the causes of paraproteinemic hemoblastoses.
Reasons
The main causes of the development of pathology:
- Ionizing radiation.
- Chemical mutagens.
- Viruses.
- Hereditary factor.
Diagnosis for paraproteinemic hemoblastoses
Pathology is diagnosed with:
- Laboratory blood test. Hemoglobin, blast cells in the blood will be reduced, the level of leukocytes, ESR, and platelets will be increased.
- Laboratory studies of urine.
- Biochemistry of blood mass for electrolytes, uric acid elements, creatinine and cholesterol.
- Laboratory study of feces.
- X-ray withfocusing on the lymph nodes, which will be enlarged.
- Ultrasound of internal organs.
- ECG.
- Virological diagnostics.
- Bone marrow trephine biopsy or lumbar puncture.
- Puncture of lymph nodes.
- Studies of cellular bone marrow composition.
- Cytological diagnostics.
- Coagulograms.
Treatment
Treatment includes chemotherapy, radiation exposure and extracorporeal blood purification. Chemotherapy is the main type of therapy for hemoblastoses. The specific drug is selected depending on the tumor process. The latest generation of cytostatics such as "Sarcolysin" or "Cyclophosphan" are used. Also suitable are Vincristine, Prednisolone, Asparaginase and Rubomycin. A bone marrow transplant, which is performed in case of remission, can permanently get rid of the disease.
Prevention
It is necessary during the remission of paraproteinemic hemoblastoses to prevent exacerbations, first of all, to exclude the means that cause them. Prevention of Rh immunization - caution in blood transfusions when Rh-positive blood is mistakenly injected. Exacerbations are also prevented with the help of a long but weak cytostatic effect.
We reviewed the main paraproteinemic hemoblastoses.
