Among the many varieties of blood pathologies, there are also those in which lymphosystemic tumors are actively formed, synthesizing immunoglobulin( or paraprotein).
All cells of the formed tumor formation evolved from a single malignant and genetically anomalous cell, and therefore they produce only one specific type of immunoglobulin.
What is paraproteinemic hemoblastosis?
In general, paraproteinemic hemoblastoses represent a group of pathological conditions in which increased production of paraproteins is observed.
Gradually, the accumulation of these immunoglobulins occurs in the blood, which adversely affects the body.
Such a reaction is caused by reduced production of other types of immunoglobulins, which leads to immunologically caused failure of the patient's body.
Species
Specialists distinguish four varieties of paraproteinemic hemoblastoses:
- Cutaneous lymphocytoma;
- Syndrome of heavy chains of Franklin;
- Waldenstrom Macroglobulinemia;
- Myeloma pathology.
Myeloma disease
Similar pathology refers to lymphoproliferative diseases in which blood cells produce an immunoglobulin of monoclonal origin.
This form of the disease usually affects patients after 40 years of age. A similar pathology usually develops after radiation exposure. It can develop for a long time secretly, but when pathological processes reach a serious stage, then there is pathological weakness, ossalgia and weight loss.
Along with this, patients are concerned about signs of a neurological nature such as fatigue and headaches, eye lesions and other visual disorders, numbness and tingling, problems with sensitivity.
The modern approach to therapy provides prolongation of life to patients for up to 4 years. If you neglect treatment, the patient will not live more than 1-2 years. In general, the prognosis depends on the patient's sensitivity to cytostatic drugs and on the characteristics of the pathological process.
Waldenstrom Macroglobulinemia
This type of paraproteinemic hemoblastosis is a bone marrow tumor, formed from lymphocyte cell structures. The general picture of Waldenstrom's macroglobulinemia consists of symptoms like:
- Arthralgia;
- Hyperopia;
- Excessive weakness;
- Long-term minor hyperthermia, which is characterized by a temperature of about 37.5 ° C;
- Sensations of heaviness to the left due to splenomegaly, and on the right due to augmentation of the liver;
- Slimming and enlarging the lymph nodes.
Also for macroglobulinemia is characterized by the presence of traditional hemorrhagic symptoms, increased viscosity and viscidity of blood, etc.
Most often, a similar form of paraproteinemic hemoblastosis develops as a result of the viral-genetic factor.
According to this theory, some viral agents after penetrating the body cause an immunodeficiency state, seep into blood and bone marrow cells and provoke them uncontrolled division, producing abnormal cells.
The genetic factor is associated with a hereditary predisposition to this pathology, because cases of familial pathology are known. Treatment is carried out by traditional methods such as chemotherapy, radiation, hemodialysis or plasma plasmapheresis, bone marrow transplantation.
Severe Franklin Disease
This form of hemoblastosis is very rare. For today in the history of medicine there are no more than a hundred cases of the disease.
It is characterized by an increase in the number of cellular structures controlling the synthesis of immunoglobulin heavy chains. Patients are concerned:
- Fever;
- Increase of the sky and tonsils;
- Increased thyroid, liver, spleen;
- Significant swelling of the lymph nodes leading to their dysfunction;
- Developed in the absence of infectious resistance acquired immunodeficiency or AIDS.It is this complication and its typical consequences that most often cause death.
Franklin's disease is characterized by a symptomatic pattern, similar to other lymphopopular pathologies. As a result of cellular changes, an abnormal immunoglobulin is synthesized, which causes the development of specific symptoms and complications. To such a disease, children and men are more likely to be younger than 40 years of age.
Lymphocytoma of the skin
For a similar type of hemoblastosis, the development of lymphatic cutaneous infiltration is typical, which is manifested by itching sensations, erythema-like eruptions. The epidermis begins to exfoliate, then tumor growths appear on this site. Usually, the tumor prefers localization in the region of the legs, on the back or face.
Skin processes may be the beginning of leukematous processes, although they often develop in parallel with bone marrow anomalies. When the pathology reaches the stage of active development, symptoms are observed:
- Erythrodermal syndrome with characteristic swelling;
- Diffuse alopecia;
- Lamination and dystrophic changes of nail plates;
- Infiltrative skin lesions, especially on the face, causing severe itching.
Plasma contains lymphocytes resembling atypical cells. Infiltration can also have an intrinsic character, affecting the kidneys and liver, which is fraught with a lethal outcome.
Common signs and causes of
Hemoblastosis is characterized by profuse symptoms of local and systemic origin. In general, the manifestations can be acute, reminding of the symptoms of a respiratory viral infection, or proceed latently, accompanied by blurred and unexpressed signs.
The first symptoms of hemoblastosis are manifestations of nonspecific poisoning, such as:
- of causeless long-term hyperthermia,
- hyperdrug,
- excessive fatigue,
- asthenic signs, etc.
Along with the listed signs, hemoblastosis is characterized by an increase in lymph nodes, as well as splenomegaly and hepatomegaly. In connection with the increase in bone marrow substance, the patient suffers severe bone pain.
There is also hemorrhagic symptomatology like subcutaneous hemorrhages, increased predisposition to bleeding from the gums, in the organs of the digestive tract, nosebleeds, etc. e
Against the backdrop of tumor processes, a secondary immunodeficiency develops, which causes the patient to lose infectious resistance. Against this background, such patients are easily affected by nosocomial infections, septic diseases, pneumonia. And these pathologies are capable of lightning fast development and a course that ends lethal.
Diagnostics of
Diagnostic measures for paraproteinemic hemoblastoses involve step-by-step implementation of the following procedures:
- Laboratory blood test. For paraproteinemic hemoblastosis, a typical low hemoglobin content, down to anemia, blast cells, elevated levels of leukocyte elements, increased ESR, increased platelet count;
- Laboratory tests of urine;
- Biochemistry of blood mass for the content of electrolytes, uric acid, creatinine and cholesterol;
- Laboratory study of feces;
- Carrying out of radiological research with an emphasis on lymph nodes, which with proteineemic hemoblastosis will have increased sizes;
- US of internal organs( spleen, heart, liver), electrocardiogram;
- Virological diagnosis;
- Bronchial trepanobiopsy or lumbar puncture;
- Puncture of lymph nodes;
- Study of cellular bone marrow composition;
- Cytological diagnosis;
- Study of the coagulation capacity of blood( coagulogram).
Treatment of
When choosing therapeutic tactics, the specialist is guided by the indicators of the patient's general health, the principles of the developed tumor process and prognostic data. In general, the treatment is based on techniques such as chemotherapy, radiation treatment and extracorporeal bleeding.
Usually the latest generation of cytostatics is used, which provides the greatest possible therapeutic effect with minimal adverse events. The intake of drugs of this type is of a course character, and the number of courses depends on the intensity of development of oncology. The most commonly used drugs today are Sarcolysine or Cyclophosphamide.
There is a specialized protocol according to which at the initial stage of treatment patients are prescribed drugs like Vincristine, Prednisolone, Asparaginase and Rubomycin. The second stage of therapy shows the use of Methotrexate, Cytosar, Cyclophosphamide. Additionally, at the second stage of treatment, the protocol recommends the use of drugs like Adriamycin, Dexamethasone, Asparaginase, Vincristine, etc.
The use of radiation therapy involves the use of ionizing effects on tumor sites in the spleen, liver or bone tissue. Extracorporeal bleeding is prescribed for the purification of blood from an excessive number of paraprotein accumulations. The most highly effective variants of this practice are hemodialysis and plasmapheresis.
It is possible to completely get rid of paraproteinemic hemoblastoses only by transplanting the medullary substance, which is performed when a stable remission is achieved.