What is myelodysplastic syndrome?

  I. Overview: Myelodysplastic syndrome (MDS) is a heterogeneous clonal disease occurring in the multipotent hematopoietic stem cell stage, characterized by irreversible quantitative and qualitative abnormalities of hematopoietic cells, manifesting as anemia, infection or bleeding and a high rate of leukemic transformation. As a common disease of the hematological system, its incidence, clinical detection rate is increasing. In recent years, there are clinical retrospective studies of myelodysplastic syndrome (MDS), the overall MDS conversion rate is only about 30%, and most MDS patients die not from leukemia, but from infections, bleeding and transfusion-related diseases due to hematocrit reduction. Anemia is the most common clinical manifestation of MDS, and long-term blood transfusion is particularly stressful for individuals, families, and society in MDS patients.  According to WHO staging criteria, MDS can be classified into refractory anemia (RA), refractory anemia with ring iron granulocytes (RARS), refractory thrombocytopenia with multilineage developmental abnormalities (RCMD), RCMD with ring iron granulocytes (RCMD-RS), refractory anemia with primocytosis-I (RARB-I), refractory anemia with primocytosis-II (RARB-II), and refractory anemia with primocytosis-I (RARB-II). II (RARB-II), 5q- syndrome and those who cannot be clinically classified. Modern medicine proposes a variety of treatments such as stimulation of hematopoiesis, induction of differentiation, low-dose chemotherapy, cytokines, and immunomodulatory therapy, but the clinical efficacy of each type varies greatly.  In the past 10 years, Chinese medicine has begun to study the etiology and treatment of this disease, especially the combination of Chinese and Western medicine has achieved certain efficacy. In recent times, many doctors have classified this disease as “deficiency labor”, “acute consumption”, “blood evidence”, “internal injury and fever”, “fever”, and “fever of temperature” in Chinese medicine. It is also classified as “Fever from internal injury” and “Fever from ambient temperature”. The etiology and pathogenesis of the disease are believed to be due to congenital deficiency of endowment, loss of nourishment, emotional and mental disorders, and poor diet, resulting in deficiency of the spleen and kidneys and depletion of the source of blood production, or external exposure to the six evil spirits, or injury caused by evil toxins that enter the internal heat, burning the blood channels and depleting the fluids, resulting in deficiency of both qi and yin and internal obstruction of blood stasis, resulting in a wide range of symptoms. The disease process can also be seen in the deficiency of both yin and yang, the failure of qi to take in blood, the deficiency of yang and blood loss and the death of yin and yang. The lesions are in the kidney and the spleen, and the deficiency is mostly seen in Qi and blood deficiency, spleen and kidney Yang deficiency, liver and kidney Yin deficiency; the actual symptoms are mostly seen in heat and toxicity, blood stasis and stagnation.  In recent years, according to WHO’s risk classification of MDS, we have been treating MDS in stages according to the pathogenesis of “deficiency of essence, internal embedding of evil and toxicity, and the axis of positive and negative effects”, combined with its pathogenetic characteristics and clinical typing, and achieved certain clinical efficacy. In clinical treatment, we found that patients in the low-risk stage of MDS are mostly seen in patients with deficiency of spleen and kidney leading to deficiency of yin and loss of yang and failure to transform essence and blood; patients in the middle-risk stage are mostly seen in patients with deficiency of qi and yin leading to deficiency of liver and kidney and internal heat toxicity; patients in the high-risk stage of MDS are mostly seen in patients with deficiency of qi and yin leading to internal stasis toxicity.  1, low-risk stage: patients clinically diagnosed with MDS, RCMD, RA, RAS and other low-risk types, and even some patients with intermediate-risk type-1, mostly without obvious chromosomal changes or good chromosomes, primitive cells < 5%, clinical features are mainly anemia, may be accompanied by irregular fever, bleeding symptoms are mild, see the face with atrophy or with grayish dullness, tiredness, palpitations and dizziness, lumbar spine soreness and weakness of both feet. There is fever from time to time, or fever in the heart of the hands and feet, thin white coating, light tongue or teeth marks, and weak pulse. In Chinese medicine, the symptoms are mostly due to deficiency of both spleen and kidney, deficiency of qi and yin, loss of yin and yang, and lack of transformation of essence and blood. Spleen deficiency is a lack of source of Qi and Blood biochemistry, see the face is atrophy or with gray dull, tiredness and reduction; kidney stores essence, the main bone marrow, essence and blood are the same source, kidney deficiency is not to fill the essence of the marrow to nourish, essence can not turn blood is not to fill the internal organs and veins, see the waist and spine soreness, both feet atrophy; Qi deficiency can not turn essence, blood deficiency is the heart loss of nourishment, see palpitations and dizziness; Yin and Blood deficiency, sometimes fever, or hand and foot heart heat. Treatment is to benefit Qi and nourish Blood, tonify the spleen and kidney, fill in the marrow, the formula uses raw roasted Astragalus, Radix Codonopsis, Radix Rehmanniae, Radix Angelicae Sinensis, Cornu Cervi Pantotrichum, Fried Eucommia, Rhizoma Atractylodis Macrocephalae, Radix Paeoniae Alba, Semen Cuscutae, Rhizoma Polygonati, Radix Aconiti, Citron Peel, Radix Scutellariae Sinensis, etc. plus or minus; even some patients may experience fatigue, less breath and lazy speech, white face and light white, fear of cold extremities, poor appetite and loose stools, soreness and weakness of the waist and knees, or swelling of the face and feet, pale and fat tongue. White tongue coating, sunken pulse and other symptoms. The clinical diagnosis is a deficiency of Yang in the spleen and kidney, which can be accompanied by warming and tonifying drugs such as Xian Ling Spleen, Boneset, Shu Shu Pian Pieces and Deer Antler Pieces, which can often be effective. At this stage, we hope to stimulate normal residual hematopoietic stem/progenitor cells, thus increasing the number of blood cells, getting rid of blood transfusion as soon as possible and improving the quality of life. A large amount of clinical pharmacology has confirmed that Chinese herbal medicines for strengthening the spleen and tonifying the kidney have the effect of promoting the proliferation and differentiation of bone marrow stem cells and early cells and self-replication, while Chinese herbal medicines can improve the imbalance of hematopoietic stimulating factors and hematopoietic inhibiting factors, reduce the apoptosis of hematopoietic progenitor cells and promote the proliferation and differentiation of early hematopoietic cells through overall diagnosis and treatment.  2.Mid-risk stage: Most of the patients in the mid-risk stage are RAEB, mid-risk-2 patients. In addition to anemia, the clinical condition is characterized by fluctuating heat patterns, or even high fever, with skin and mucous membrane bleeding, primitive cells > 5%, or even peripheral blood with various types of infantile cells. The evidence belongs to deficiency of Qi and Yin, heat and toxin induce liver fire, the pathogenesis of Chinese medicine is mostly based on poison and stasis, the positive Qi is weak, Qi and Yin are deficient, the evil heat is internalized and invades the bone marrow, for a long time it consumes the body’s essence and blood, resulting in the body’s essence deficiency and blood deficiency, the internal organs are deficient; the evil of heat and toxin evaporates outward from the bone marrow, soaks the organs, often induces the liver fire, internally offends the blood, damages the ligaments and veins and sees bleeding, it is mostly a mixed evidence of deficiency and reality, as the saying goes “The treatment is to strengthen the spleen and nourish the kidneys, clear the liver and detoxify the blood, drain the heat and stop the bleeding, using princeton ginseng, fried atractylodes, raw peony, large shengdi, cyperus root, danpi, cypress, raw acacia flower, dried foxglove, roasted licorice, poria, Chenpi, plus or minus water hyacinth, dry lotus grass, The patient is often quicker to move backwards. Patients at this stage often develop more quickly, so although the deficiency evidence is heavy, the effect of using Qi and nourishing Yin alone is often poor, so do not dare to omit the anti-cancer drugs to clear heat and detoxify, mostly use Qing Dai, Flea Huo, Vine root, or add Niuhuang detoxification tablets, etc.; if bleeding is not obvious, you can add drugs to activate blood circulation and remove blood stasis, such as Trigonella, Curcuma longa, Tiger Balm, etc. Clinical use of small doses does not aggravate the worry of bleeding. For patients in this stage, we use heavy medicine to detoxify the toxin and save the marrow; and the drugs that benefit qi and nourish yin can improve the body’s own immune function, and the drugs that activate blood circulation and remove blood stasis have the effect of regulating immune function, improving the state of immune dysfunction, increasing the sensitivity of red lineage progenitor cells to erythropoietin, making red blood cells proliferate and differentiate, and treating the symptoms to support the root, enhance the body’s own anti-tumor vitality, kill tumor cells, and improve It is hoped that the treatment can strengthen the body’s own anti-tumor vitality, kill tumor cells and improve anemia, so that the disease can be relieved and enter the low-risk stage.  High-risk stage: Patients in the high-risk stage are mostly seen in relapsed and refractory MDS, high-risk MDS, most of them have clear basic leukemia symptoms, and patients in this stage often meet the diagnostic criteria of leukemia when they have bone puncture in different parts or have bone puncture again within a short period of time. Patients with deficiency of Qi and Yin for a long time resulting in internal stagnation of toxins, and patients with increased symptoms of Qi and Yin deficiency after chemotherapy, mostly combined with liver fire and heat, so the clinical evidence is mostly to benefit Qi and nourish Yin, to support the righteousness and eliminate evil, and also to clear the fire of liver wood, with the use of Prince’s ginseng, Fu Ling, Atractylodes macrocephala, Bai Shao, Tian Dong, Sheng Di, Huang Bai, Bei Sha Shen, Mai Dong, whole Angelica, Fructus Lycii, Chen Pi, Dandelion, Serpent’s tongue grass, tiger root, sheep’s foot root, raw malt, raw chicken gold Add and subtract. When the condition is stable, the treatment can be strengthened to strengthen the spleen, nourish the kidney and clear heat from the liver, and the medicine can be changed to raw astragalus, princely ginseng, fried atractylodes, made of semen, angelica, fried eucommia, Chinese hyssop, wolfberry, snake tongue grass, Chen Pi, raw roasted grass, tiger stick root, cyperus root, sheep’s foot root, chicken blood vine, codonopsis, raw white peony, bone marrow, dandelion, fried cyperus, etc. plus and minus. At this stage, the patient’s condition is critical, deficiency and evil toxicity, the prognosis is poor, the combination of Chinese and Western treatment, in killing the malignant clone, restore normal hematopoietic function, but still do not forget to regulate the internal organs, support the qi and blood, remove the remaining evil, in order to consolidate the effect of treatment, to obtain long-term remission.  4, add or subtract with the symptoms: the symptoms of fever should be distinguished from external sensation, heat, the difference between bone steam and labor heat. For external sensation, we can add Yinhua, Forsythia, Thornbush, Bupleurum, Chaihu, Da Qing Ye, etc.; for internal fever with increased leukocytes, we can add Baihua Serpent’s Tongue and Tongue Herb, Bai Ying, Shan Dou Root, Da Qing Ye, etc. to clear volatile toxins; for bone vapor and labor fever, we can add Artemisia, Dioscorea, Turtle A, etc. In case of actual bleeding, we can use Cyperus roots, Buffy’s horn, Danpi, Fenugreek, etc. to clear heat toxins and cool the blood to stop bleeding; in case of deficiency, we can use Dragon Bone, Oyster, Cornu Cervi Pantotrichum, Ginseng Sanqi, etc. to consolidate and stop bleeding. The spleen and stomach are the basis of postnatal biochemistry, and they nourish the qi and blood. At the same time, attention should be paid to regulating the liver, soothing liver qi, softening liver yin and clearing liver fire.  In 1997, the International Symposium on Prognostic Analysis of MDS selected the three parameters with the strongest prognostic significance, namely the percentage of bone marrow primitive cells, karyotype and peripheral cytopenia series, and developed an International Prognostic Score System (IPSS), which classified MDS into four risk groups, including low-risk, intermediate-risk-I, intermediate-risk-II and high-risk. The usefulness of the IPSS for suggesting prognosis and guiding treatment of MDS has been confirmed in subsequent studies by different authors. To date, the IPSS has largely replaced the previous prognostic systems and is widely accepted by hematologists in various countries. Modern medicine has been unable to effectively resolve the contradiction between the body’s protective mechanism leading to the inhibition of malignant clone proliferation and the consequent excessive apoptosis, and the contradiction between autoimmune damage and immune compromise leading to malignant clone proliferation in the study of the disease mechanism of MDS, which also suggests that clinical suppression of immunity alone or without selective suppression of immunity is detrimental to the treatment of the disease and aggravates It also explains why clinical use of immunosuppressants and the continuous introduction of a variety of immunosuppressants, but the clinical efficacy is not yet satisfactory.  In our clinical work, according to the risk classification of MDS, the characteristics of miscellaneous evidence and changes in each stage of the disease, and the trend of positive and evil, we combine evidence and disease identification, benefitting Qi and nourishing Yin to support the positive, and at the same time, adding appropriate anti-tumor activity of In the treatment of low-risk patients, it is common to add heat-clearing and detoxifying herbs with anti-tumor activity. In the treatment of low-risk patients, we can often boldly use flesh and blood products to warm the kidney and fill the marrow; while in the diagnosis and treatment of medium-risk patients, detoxification and marrow rescue should be the top priority, taking into account the support of the righteousness, strengthening the spleen, benefiting the kidney, invigorating the blood and resolving blood stasis, and treating patients with evidence. At the same time, the combination of Chinese herbal medicine and Chinese and Western medicine has obvious advantages in the development and prevention of recurrence of the disease, as well as in the pre-treatment of the disease, especially in the disorder of the immune system, cell repair, antioxidation, anti-mutation, anti-apoptosis, and receptor sensitization. In the current situation of imperfect disease diagnosis and treatment, Chinese herbal medicine and the combination of Chinese and Western medicine have an irreplaceable position.