In accordance with ICD-10, paraproteinemic hemoblastosis is assigned to class 2 neoplasms (C00 — D48), part C81 — C96. This includes malignant tumors of the hematopoietic, lymphoid and tissues related to them.
They belong to the group of tumor diseases of the circulatory system, the main feature of which is the secretion of paraproteins or / and their fragments. In different patients, paraproteins can reach significant concentrations in the blood serum and belong to different classes. The source of tumor growth is B-lymphocytes.
Paraproteinemic hemoblastoses are common throughout the world. As the patient ages, their frequency only increases.
Classification of paraproteinemic hemoblastoses
The forms of hemoblastoses are distinguished depending on which immunoglobulins they secrete, and on what morphological characteristic the tumor substrate:
- lg-secreting lymphomas;
- multiple myeloma;
- heavy chain diseases;
- acute plasmoblastic leukemia;
- Waldenstrom macroglobulinemia;
- solitary plasmacytoma.
General clinical signs
What are the symptoms of paraproteinemic hemoblastosis?
The clinical picture is characterized by the presence of a tumor, which produces paraprotein, and secondary humoral immunodeficiency, which develops in all patients when an increase in tumor mass occurs. By the nature of the course of the disease, the chronic (unfolded) and acute (terminal) stages are distinguished.
Paraproteinemia causes common manifestations for p.
- Peripheral neuropathy.
- Increased blood viscosity.
- Microcirculatory disorders.
- Hemorrhagic syndrome.
- Damage to the kidneys.
- Cryoglobulinemia of 1-2 types, amyloidosis.
This is the most common classification of paraproteinemic hemoblastoses. Consider each view in more detail.
Multiple myeloma
Multiple myeloma is considered the most common P. g. With unclear reasons for its development. The morphological picture is represented by plasma cells of a certain degree of maturity, often having atypical features. The expanded stage is characterized by the localization of the tumor in the bone marrow, sometimes in the lymph nodes, spleen, and liver.
The nature of the distribution of foci in the bone marrow allows us to distinguish several forms of multiple myeloma: diffuse focal, diffuse and multiple focal.
Around the tumor foci, the bones are destroyed because multiple myeloma stimulates osteoclast activity. The osteolytic process in various forms has features. For example, a diffuse focal form is characterized by osteoporosis, causing the appearance of foci of osteolysis; diffuse - observation of osteoporosis; multiple focal - possible individual osteolytic foci. The expanded stage of the tumor usually does not affect the destruction of the cortical layer of the bone. She thins it and raises it, forming at the same time on the skull, sternum, ribs of a swelling. The terminal stage of the tumor is characterized by the formation of end-to-end defects and germination in the soft tissues that are 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-secretory.
Myeloma is divided into 3 stages, based on an analysis of blood creatinine, hemoglobin, urinary and serum levels of paraproteins, and x-rays of bones.
- Stage 1 - the tumor has a mass of less than 600 g / m 2 .
- Stage 2 - from 600 to 1,200 g / m 2 .
- Stage 3 - more than 1,200 g / m 2 .
The absence or presence of renal failure affects the assignment of the stage of the symbol A or B. What are the symptoms of this form of paraproteinemic hemoblastosis?
The tumor has a diverse clinical picture. The appearance of the first signs usually occurs in the third stage (weakness, fatigue, pain). A consequence of the osteodestructive process is the development of pain. The most common pain is damage to the sacrum and spine. Quite often, the ribs, parts of the humerus and femur, affected by the tumor, are sore. Extradural localization of foci is characterized by the fact that compression of the spinal cord follows a pronounced pain syndrome.
The diagnosis is established on the basis of data obtained after sternal puncture and detection of monoclonal immunoglobulins in urine and / or blood serum. X-ray examination of the skeleton has additional value. If the patient has a multiple-focal form, then the sternum puncture may not reveal the tumor.
After the diagnosis of paraproteinemic hemoblastoses (a photo of the tumor can be found in the article) has been established, an X-ray examination of the skeleton is carried out before treatment, and liver and kidney function are checked. The use of excretory urography and other aggressive methods for examining the kidneys is not allowed, since they can provoke the development of their irreversible acute failure.
Myeloma Therapy Methods
As a rule, they begin treatment with a hematological hospital, and then it can already be carried out on an outpatient basis.
In cases where a separate foci of osteolysis creates 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 after decompression laminectomy, radiation therapy is recommended.
Solitary plasmacytoma
What other paraproteinemic hemoblastoses are there?
Solitary plasmacytoma is a local tumor. The clinical picture is dependent on size and location. Most often, solitary plasmacytomas are multiple myeloma at an early stage. Bone solitary plasmacytoma is prone to generalization, revealed as multiple myeloma 1-25 years after the radical therapy was performed.
Extrabone 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 data from morphological examinations of biopsy or puncture material. Before starting treatment, it is necessary to exclude multiple myeloma. Radical surgery and / or radiation therapy are used for treatment, with which 50% of patients are completely cured. Patients with solitary plasmacytoma are under observation for life due to the fact that a generalization of the process is possible.
This is not all types of paraproteinemic hemoblastoses.
Waldenstrom macroglobulinemia
Waldenstrom's macroglobulinemia is chronic in most cases, subleukemic or aleukemic lymphocytic leukemia. Localization of the lgM-secreting tumor occurs in the bone marrow. Characterizes its lymphocytic composition of cells with an admixture of plasma. In addition to monoclonal IgM, Bens-Jones protein is secreted by tumor cells in approximately 60% of patients. Waldenstrom's macroglobulinemia is much less common than multiple myeloma.
The most common clinical manifestations include bleeding and high viscosity syndrome. You can also observe peripheral neuropathy, secondary immunodeficiency, amyloidosis, kidney damage. Renal failure rarely develops. For the advanced stage, an increase in the liver, spleen and / or lymph nodes, and weight loss in 50% of patients are characteristic. Anemia develops late, white blood cells may be normal, white blood cell count unchanged, lymphocytosis with moderate leukocytosis is quite common, some neutropenia is possible. A common occurrence is a sharply increased ESR.
The diagnosis is made on the basis of the immunochemical determination of monoclonal IgM in the blood, data of trepanobiopsy or sternal puncture, electrophoresis of urine and serum proteins . Therapy is carried out in a hematological hospital. What else is paraproteinemic hemoblastosis?
Heavy Chain Disease
Clinical and morphological characteristics of heavy chain diseases are very diverse. Their features include the presence of an abnormal protein in the urine and / or blood serum. Heavy chain a-, g-, m-diseases are distinguished.
The most common a-disease, affecting mainly children and young people under the age of 30 years. The disease is common in the Middle and Middle East, in the countries of the Mediterranean basin. It has two forms of course: 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 heavy chain disease g (Franklin's disease) is found in only a few dozen patients. Morphological data and the clinical picture are diverse, not very specific. Proteinuria, relative neutropenia, thrombocytopenia, progressive anemia, abnormal fever, damage to the Waldeyer ring with swelling of the tongue, soft palate and erythema, enlargement of the liver, lymph nodes and spleen are most often noted. This disease is usually fast progressing and difficult. Within a few months, death occurs.
Heavy chain disease m is the rarest form. As a rule, the elderly are susceptible to the disease. The disease manifests itself in the form of sub-leukemic or aleukemic lymphocytic leukemia, usually the increase in lymph nodes is not pronounced, but the spleen and / or liver are enlarged. Amyloidosis and osteo-destruction are noted in some patients. 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.
Clinical diagnosis is extremely difficult. Its establishment occurs through immunochemical methods, which detect heavy immunoglobulin a-, g- or m-chains in urine and / or blood serum. Treatment of paraproteinemic hemoblastoses of this type takes place in a hematological hospital.
lg-secreting lymphoma
lg-secreting lymphoma is a tumor that has predominantly extramedullary localization, often they are highly differentiated (lymphoplasmacytic, lymphocytic), rare-blast, i.e. sarcomas. The difference from other lymphomas is the secretion of monoclonal immunoglobulins, often M-class, a little less often than class G and very rarely A, including Bens-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 with Waldenstrom macroglobulinemia and multiple myelomas.
Next, we will examine the causes of paraproteinemic hemoblastoses.
Causes
The main causes of the development of pathology:
- Ionizing radiation.
- Chemical mutagens.
- Viruses.
- Hereditary factor.
Diagnostics for paraproteinemic hemoblastoses
Pathology is diagnosed with:
- Laboratory blood tests. Hemoglobin, blast cells in the blood will be reduced, the level of leukocytes, ESR, platelets will be increased.
- Laboratory tests of urine.
- Biochemistry of blood mass on electrolytes, uric acid elements, creatinine and cholesterol.
- Laboratory study of feces.
- An x-ray focusing on the lymph nodes, which will be enlarged.
- Ultrasound of the internal organs.
- ECG.
- Virological diagnosis.
- Bone marrow trepanobiopsy or lumbar puncture.
- Lymph node puncture.
- Studies of cellular marrow composition.
- Cytological diagnosis.
- Coagulograms.
Treatment
Treatment includes chemotherapy, radiation exposure and extracorporeal blood circulation. Chemotherapeutic treatment is the main form of therapy for hemoblastoses. The specific drug is selected depending on the tumor process. The latest generation cytostatics such as "Sarkolizin" or "Cyclophosphamide" are used. Also suitable are Vincristine, Prednisone, Asparaginase, and Rubomycin. A bone marrow transplant, which is carried out in case of remission, can permanently relieve the disease.
Prevention
During remission of paraproteinemic hemoblastoses, prevention of exacerbations is necessary, first of all, the exclusion of drugs that cause them. Prevention of Rh immunization - caution in blood transfusions, when Rh-positive blood is mistakenly injected. Also, exacerbations are prevented by means of a long, but weak cytostatic effect.
We examined the main paraproteinemic hemoblastoses.