Primary myelofibrosis referred to in the ICD is a disease that predominantly occurs chronically. As a rule, the case is idiopathic. The therapeutic course is selected in such a way as to maintain the vital functions of the patient's body. A feature of the condition is overly active generation of connective tissue in the bone marrow. At the same time, the body loses the elements of the hematopoietic system, which activates the hematopoietic function outside the bone marrow.
General Information and Ciphers
It is customary to isolate secondary, primary myelofibrosis. In the ICD-10, this disease is recorded by the codes D47.4, C94.4. The first includes the main group of cases, the second is devoted to the acute form. D47.4 - ICD code for primary myelofibrosis, secondary, megakaryocytic, chronic idiopathic. It was revealed that this pathological condition can develop against the background of benign, malignant neoplasms and blood diseases.
If primary myelofibrosis is diagnosed, the ICD-10 code mentioned in the patient card is D47.4. Responsibility for the correct selection of encoding rests with the attending doctor. The importance of fixing the correct code is associated with the need for official registration of all stages of patient monitoring. D47.4 - ICD-10 code for oncological primary myelofibrosis, but at the same time an idiopathic form of the disease and secondary pathology. In the patient’s card, the attending doctor specifies, writing in a specific code, what exactly is hidden under it.
Where did the trouble come from?
It is known that with primary myelofibrosis, pathological conditions associated with the cellular structure of the bone marrow are often also recorded in the patient’s medical history. Stem cells of an organ from the polypotent category can undergo malignancy, which leads to the pathology in question. In clinical practice, its frequency of occurrence is significantly higher than secondary cases of the disease. Polypotent cell structures stimulate the production of abnormally large amounts of collagen by bone marrow fibroblasts. Collagen producing cells are normal in myelofibrosis, not transformed.
Primary myelofibrosis is accompanied by the release of numerous normoblasts and granulocytes into the circulatory system. A blood serum test shows an excess of normal LDH concentration. Gradually, this leads to a lack of functioning of the bone marrow, as a result of anemia, thrombocytopenia. On average, in one out of ten cases, the disease proceeds aggressively, progresses rapidly and does not respond to therapeutic programs using even the most modern means.
Case Nuances
Primary myelofibrosis leads to bone marrow fibrosis, the patient is diagnosed with hematopoiesis in extramedullary form, which means the generation of blood cells outside the bone marrow structures. A blood test shows a leuko-erythroblast picture. At first, the patient is diagnosed with hypercellular bone marrow. The study of the cellular composition allows you to determine the increased content of pathological changes in megakaryocytes. These cells are responsible for the generation of growth factors, their delivery to the nearest tissues responsible for hematopoietic function. Excess leads to reactive reproduction, proliferation of fibroblasts.
How to notice?
With primary myelofibrosis, the prognosis and choice of a therapeutic program largely depends on how quickly an accurate diagnosis can be made. In an impressive percentage of cases, the pathological condition does not at all manifest itself with specific symptoms. If there are any phenomena indicating a pathology, then most often it is anemia, splenomegaly. If the disease has developed to a late stage, the patient as a whole feels weak, he is worried about unreasonable malaise, fever, weight loss. Possible splear infarction. The main percentage of patients observed over time suffers from hepatomegaly.
More often, treatment of primary myelofibrosis is required for people over the age of fifty. Patients seek medical attention due to lethargy, weight loss and active sweat secretion at night. Instrumental examination may show a significant increase in the spleen. The phenomenon is explained by extramedullary hematopoiesis.
Status Update
If treatment of primary myelofibrosis is started in time, the prognosis of the case will be relatively positive. To do this, you need to go to the clinic, as soon as the dubious symptoms begin to disturb, as well as insist on a detailed examination if it is not possible to determine the cause of weakness and anemia. As a rule, the suspicion of the pathology under consideration decreases with an increase in the content of LDH in the circulatory system, with the detection of splenic infarction and splenomegaly.
Quite characteristic for different stages of primary myelofibrosis, anemia will be a phenomenon, the symptoms of which are gradually becoming more pronounced. Blood tests show a variety of morphology. As a rule, red blood cells differ from each other in shape; the variety is great. In some patients, polychromasia, reticulocytosis are detected. A characteristic characteristic of the pathological condition under consideration is dacryocytes. The circulatory system contains red blood cells with nuclei, neutrophilic precursors are found. The leukocyte concentration is higher than normal, but it is impossible to talk about the average value - the variability is extremely high. A low white blood cell concentration indicates a severe case and an unfavorable progression scenario. The main percentage of detected neutrophils is immature cell structures.
Diagnostic nuances
Sometimes in clinical practice there are cases of primary myelofibrosis, the clarification of which is associated with a number of difficulties. When in doubt about the final diagnosis, the concentration of CD34 + -type cell structures in blood samples obtained from the peripheral system should be detected. With the disease in question, the content will significantly exceed the norm.
Marrow aspiration rarely results in successful acquisition of specimens for research. To accurately formulate the diagnosis, tissue samples are required for histological laboratory testing in order to confirm fibrosis. Take into account that such may be uneven. The best case assessment method is trepanobiopsy. The doctor’s task is to obtain tissues in the study of which the data will be sufficient to determine the patient’s condition.
Blood analysis
In primary myelofibrosis, most patients have leukoerythroblastic anemia. In the circulatory system, an active circulation of immature erythroid cells, myelocytes is detected. In some patients, tear-shaped poikilocytosis and giant platelets are found. The leukocyte concentration may decrease, in other cases - increased. Platelet concentration varies greatly - from less than or exceeding the norm to the standard.
The pathological condition under consideration is accompanied by active cellular decay, due to which the concentration of urates increases. Most patients have a lack of folate in the body. Marrow aspiration is a problem; trepanobiopsy can detect an increased content of megakaryocytes, reticulin. According to the results of the analyzes, it is seen: bone marrow tissue is gradually replaced by fibrous.
What to count on?
With primary myelofibrosis, life expectancy varies significantly from case to case. A five-year period is considered a median, but certain factors may worsen such a forecast. In addition, it is far from always possible to identify a pathological condition at a fairly early stage. The prognosis is worse if the hemoglobin content is less than 10 g / dl. Previously, the patient underwent a blood transfusion. Leukositosis, leukopenia indicate poor prognosis. With primary myelofibrosis, life expectancy will be less if the content of platelets in the circulatory system varies less than 100,000 units / µl. For such a risk group, it is predicted that the average life expectancy is a year, sometimes even less.
The pathological processes that cause the disease are not amenable to control methods and methods, they cannot be reversed. The most advanced treatment for primary myelofibrosis involves an allogeneic transplant of bone marrow stem cell structures. Today, this method is the only one that allows you to count on a longer life expectancy. On average, the disease lasts 1-20 years, some patients develop an even longer period. From the moment of making an accurate diagnosis, the average life span of a patient is four years.
Therapeutic measures: what will help?
Treatment is chosen based on the stage of primary myelofibrosis. The main objective of the therapeutic course is to alleviate symptoms and minimize the severity of complications. Palliative treatment involves the intake of androgens, a chemotherapeutic program, splenic embolization, and ionizing radiation. If at certain hemoglobin levels in the circulatory system the erythropoietin content is lower than normal, it can be administered artificially, thereby adjusting the hemoglobin concentration as well. In clinical practice, cases of successful allogeneic transplantation of bone marrow cells without organ ablation have been recorded. Typically, such an event is indicated for persons under 65 years of age, but there have been cases of the application of the method for older patients, and the outcome of the transplantation was satisfactory.
Presumably, drugs that inhibit the JAK cascade can be used. Scientists believe that such tools will help to control splenomegaly and pathological transformations of peripheral blood at the cellular level. Currently, this group of medicines is still under development.
Nuances and features of treatment
The main course of treatment is associated with the control of the symptoms of the disease. In particular, the correction of anemia is possible due to erythrocyte transfusion, and a lack of folic acid is leveled by special nutrition. In primary myelofibrosis, food additives containing essential components are indicated.
The use of hydroxycarbamide helps prevent spleen overgrowth. This substance allows you to control the concentration of white blood cells, weaken the general symptoms of the disease. In some cases, the patient is shown splenectomy, if the spleen increased to too large dimensions. Sometimes splenectomy becomes the optimal solution if symptomatic pancytopenia is established. This condition may be secondary against hypersplenism.
For young patients, consider the possibility and justification of bone marrow transplantation.
About status
In primary myelofibrosis, disability can be calculated only if the diagnosis is made accurately, the malignancy of the pathological condition is proved. In this case, the status of a disabled person is assigned according to the general rules, as well as to any people suffering from cancer. At the initial stage, while there is no clarity with the definition of the disease, it is not worth claiming disability.
Primary myelofibrosis belongs to the class of deadly diseases, it provokes a fatal outcome after a fairly short time from the moment the condition is clarified, so the state provides some financial support to patients. Obtaining the status of a disabled person is possible only by decision of a specialized commission responsible for this issue. For consideration of officials will have to provide a complete list of documentation, all certificates and confirmation of the diagnosis from the clinic.
Features of the disease
At the moment, myelofibrosis is the subject of research by doctors, scientists leading world institutes. Although it is known for sure that the disease is due to the growth of a mutant clone of the hematopoietic bone marrow system, the sequence of processes occurring in the patient’s body has not been conclusively established. One of the assumptions - fibrosis becomes a bone marrow response to abnormal proliferation of cell structures. An alternative opinion is that fibrosis is a component of pathological processes. Scientists have yet to clarify all the nuances of this phenomenon. Splenic metaplasia, and in some cases similar processes in the liver and other organs, are considered to be compensatory. At the same time, it has not been proven that this is the case; there is the possibility of the formation of metaplasia due to cell proliferation.
In general, people at the age of 50-70 are considered to be at risk for the disease. Frequency and gender do not correlate.
Trivia or important?
Myelofibrosis develops very slowly, and the onset of the disease is always hidden. Patients come to see a doctor because of weakness and breathing problems, cravings for sleep. These symptoms are associated with anemia provoked by the pathological condition in question. Perhaps a slightly different course of the disease, and the first ones to manifest themselves are disorders caused by splenic growth. The patient is disturbed by bloating, after a meal there are discomfort, often tormented by heartburn. Splenomegaly can cause swelling of the ankles. In general, this symptom is characteristic of almost all cases of myelofibrosis, but the enlargement of the organ varies significantly: in some it is insignificant, in others the spleen becomes truly gigantic. With an infarction of the organ, a sharp, sharp pain occurs under the ribs in the right half of the body, a specific noise of friction.
In many patients, myelofibrosis is accompanied by hepatomegaly. There are cases when the diagnosis was made, identifying the cause of stably elevated pressure. Portal hypertension can be triggered by the formation of a thrombus in the splenic vein, extramedullary cell infiltrate, as well as increased blood filling of the portal tract. With this form of high blood pressure, the patient is prone to bleeding, ascites. With a high degree of probability, varicose veins of the esophagus veins will be detected.
Note
There are known cases when myelofibrosis was diagnosed in people who turned to the doctor because of gout symptoms. The disease can be accompanied by itching, worse when exposed to heat. The calf muscle tissue is prone to convulsions, and the bones respond with pain. The complexity of the diagnosis is that such phenomena can easily be explained by the patient’s advanced age, and this slows down the timely determination of myelofibrosis.
Platelet functional malfunctions are possible. This can cause hemorrhages inside the skin, in the stomach or intestines. Approximately one in three patients develop foci of osteosclerosis. More often pathological processes cover the axial skeleton, epiphyses of the bones of the thigh and shoulders. The skull and other skeletal elements are somewhat less likely to suffer.
Can I protect myself?
The pathology under consideration is still mysterious for doctors. It was established that the deterioration of the patient's condition is possible in contact with toluene, benzene. It has also been proven for certain that these substances cannot start a disease. This significantly complicates the possibilities of prevention - no reliable and effective measures to prevent the disease have been developed, since it was not possible to establish the exact reasons. A number of theories, assumptions require proof.
Disease phases
It is customary to distinguish three stages of a pathological condition: proliferative, sclerotic, blast crisis. The first step involves the active release of leukocytes into the circulatory system. During sclerosis, hematopoietic function is inhibited, pancytopenia appears. The last stage is the crisis.
The first step is rarely symptomatic. If the disease develops very slowly and for a long time, manifestations of signs characteristic of tumor processes are possible. The patient weakens, sometimes suffers from fever, low-grade fever. Weight loss, night sweats, severe sweating.
The sclerotic stage of the development of the disease is associated with the progress of pathology and more vivid symptoms associated with anemia. The patient becomes weaker, pain periodically disturbs the left under the ribs, often a feeling of general discomfort comes. An increase in the spleen in dimensions provokes heaviness under the ribs on the left.
The blast crisis step manifests itself as pale, yellowish skin. Palpation allows you to determine a significant increase in the spleen, at a later stage - the liver. In some patients, hypersplenism is formed due to erythrocyte death in the spleen structures.
Common symptoms
The phenomena listed below are unlikely to help suspect myelofibrosis, since they are quite general and are characteristic of a wide range of diseases, but with this diagnosis they are most likely explained to them. Primary myelofibrosis leads to yellowness of the mucous membranes. Gradually, this phenomenon becomes more and more pronounced. An examination of the blood reveals an increased rate of free bilirubin. Visually, you can notice changes in urine: the shade of the liquid gradually darkens. In addition, the progress of myelofibrosis is always associated with increased anemia.
These phenomena can cause a detailed laboratory study, even in the absence of the symptoms previously described. If the doctor suggests myelofibrosis, the patient is sent for a blood test.
They take a general analysis and bone marrow puncture, which normally cannot be obtained. Trepanobiopsy is prescribed, genetic features are investigated. As a rule, with myelofibrosis, abnormalities in the chromosomes are detected. A coagulogram is shown to evaluate platelet aggregation. Blood plasma is taken for biochemistry.