1. What is the concept of myelodysplastic syndrome?
Myelodysplastic syndrome (MDS) is a clonal disorder of hematopoietic stem cells. There are qualitative and quantitative abnormalities in blood cell production, mostly in elderly patients and rarely in children. The main clinical symptoms are anemia, sometimes with bleeding or infection, peripheral whole blood cytopenia, and both lineage cytopenias; the bone marrow is mostly proliferating or significantly active, with significant pathological hematopoiesis and increased primitive cells, which can occasionally be present in the peripheral blood. The application of general anti-anemia drugs is not easy to take effect, the course of the disease is long, and most of the amount will eventually transform into leukemia or die from the comorbidities caused by allogeneic cytopenia. In 1982, the FAB Collaborative Group first advocated the concept of MDS and made clear recommendations on the typing and diagnosis of MDS.
2. What is the etiology of MDS?
The exact etiology is generally unknown and may be related to chemical carcinogens, alkylating agent therapy or radiation exposure. The mode of progression of patients with MDS and its transformation to acute leukemia depends largely on the type and number of oncogenes activated in the cells. It is currently believed that the disease occurs as a result of clonal mutations in earlier hematopoietic stem cells, which have been damaged.
3. What are the clinical manifestations of myelodysplastic syndrome?
It is most common in people over 50 years of age and occasionally in children. Most of them have an insidious onset, with anemia as the main manifestation, which is persistent and progressive, and is not easily treated with iron or various blood-producing drugs.
4. What issues should be noted in the diagnosis of myelodysplastic syndrome?
(1) Pathological hematopoiesis is the key to the diagnosis of MDS, but it is not unique to MDS and can be seen in a variety of myeloproliferative diseases, anemia and non-hematopoietic histomas.
(2) Primitive cells are extremely important for the typing and prognosis of MDS. If the BM primitive cells in MDS are less than 5%, it is considered a low-risk type.
(3) The classification of MDS is not a separate disease entity; each type is a different stage of the same disease, a different period of progression to acute leukemia.
(4) Abnormalities of chromosomes are very important for the diagnosis of MDS.
5. What is the clinical staging of myelodysplastic syndrome?
There have been many improvements in the typing of MDS, and currently the WHO classifies MDS into the following types.
(1) Refractory anemia (RA)
(2) Refractory anemia with ringed iron granulocytes (RAS, RARS)
(3) Refractory hematocrit with multilineage disease (RCMD)
(4) Refractory anemia with primitive cellular excess (RAEB)
(5) MDS unclassifiable (MDS-U)
(6) 5q-syndrome
6. What is the differential diagnosis associated with myelodysplastic syndrome?
(1). Aplastic anemia: Aplastic anemia without pathological hematopoiesis. Clinical manifestations are characterized by a decrease in whole blood cells, low but not active bone marrow proliferation, a relative increase in lymphocytes, visible non-hematopoietic cells, and no abnormal cell morphology.
(2). Megaloblastic anemia: Megaloblastic anemia is quite similar to the megaloblastic form of this disease. However, the essence of megaloblastic anemia is folic acid and vitamin B12 deficiency. The deficiency can be treated with vitamin B12 or folic acid supplementation. In contrast, in this disease, there is much increased folic acid and vitamin B12 in the blood, and treatment with folic acid or vitamin B12 is ineffective. In terms of cell morphology, megaloblastic anemia blood picture, typical megaloblastic red blood cells in all stages are seen in bone marrow picture, and pathological hematopoiesis is not obvious.
(3). Acute leukemia: The clinical manifestations and peripheral blood changes of acute leukemia are similar to those of myelodysplastic syndrome, but the diagnostic criterion for acute leukemia is that the sum of primitive and early juvenile or naive cells in the bone marrow is greater than 30%, whereas the sum of primitive and early juvenile or naive cells in the bone marrow of patients with myelodysplastic syndrome is less than 30%.
(4). Hemolytic anemia: Refractory anemia in myelodysplastic syndrome has increased bone marrow red lineage and slightly increased reticulocytes in blood similar to hemolytic anemia, but hemolytic anemia has positive anti-human globulin test or positive acidified serum test. In contrast, myelodysplastic syndrome is negative for both of these tests, and hemolytic anemia does not have chromosomal abnormalities, whereas myelodysplastic syndrome has chromosomal abnormalities. In contrast, if the treatment of myelodysplastic syndrome is effective, the reticulocytes rise first and then the hemoglobin rises.
7. What are the advantages of combining Chinese and Western medicine in the treatment of MDS?
The basic principle of disease treatment is to treat the disease urgently and to treat the root cause slowly, combining Chinese and Western medicine, walking on two legs, double-pronged, treating both the symptoms and the root cause, on the basis of overall conditioning, stimulating normal hematopoiesis with a variety of drugs, inhibiting pathological hematopoiesis, and then exorcising the evil on the premise of supporting the righteousness to restore the normal function of the bone marrow, which is more conducive to the healing of the disease.
8. Chinese medicine’s understanding of the etiology and pathogenesis of MDS?
(A) Weak endowment and disease caused by deficiency
The parents may be weak and sickly, old and decrepit, or the loss of nourishment in the fetus, the lack of nurturing, so that the congenital deficiency, weak endowment, easy to suffer from disease, the disease is easy to form a state of prolonged illness, resulting in the internal organs of Qi, blood, Yin and Yang deficiency. The kidney is the essence of the innate nature, and it collects the essence of the five viscera and six bowels; the kidney is the main bone and marrow; the kidney collects the essence, and the essence transforms the blood; if the endowment is insufficient, there is no source of biochemistry, and thus the symptoms of qi deficiency, blood deficiency and marrow deficiency appear.
(2) Inadequate diet and damage to the spleen and stomach
The Su Wen – the chapter of determination of gas says: “The middle jiao receives gas to take juice, changes and red, is blood.” It is evident that the spleen and stomach are the origin of the latter and the source of biochemistry of qi and blood. If overeating, hunger and satiety is not regulated, eatery, malnutrition, or excessive drinking can lead to damage to the spleen and stomach, can not produce water and grain essence, then the day after the loss of nourishment, the source of Qi and blood is not full, the internal organs and meridians are not moistened, and induce the disease.
(3) Long-term illness, loss of conditioning
After a prolonged illness, loss of conditioning, prolonged illness will lead to deficiency, prolonged illness will lead to stasis, and deficiency will lead to deficiency of qi and blood, qi stagnation and blood stasis, which can be seen as phlegm nucleus, scrofula, abdominal lump, and tongue with petechiae. The Jin Plaque – deficiency labor chapter said: “five labor deficiency pole win thin, abdominal fullness can not eat, food injury, worry injury, drinking injury, room injury, hunger injury, labor injury, meridian Ying and Wei Qi injury, internal dry blood, skin nail wrong, two dark eyes.” This passage, to a certain extent, explains the pathogenesis of this evidence.
(IV) Weakness of the internal organs and invasion of evil toxins
The patient’s body is weak, and the six external evil toxins take advantage of the weakness to invade the patient. The external evil enters the interior and turns into heat, burning the fluids and blood channels, and the blood stagnates in the meridians and internal organs and becomes stasis. The abnormal metabolism of water and fluid stagnates in the meridians and veins and becomes phlegm and dampness, and phlegm and stasis intermingle, so there are dark lips and mouth, petechiae in the skin and mucous membranes, and Y lumps in the abdomen. This is what the Suwen – Legacy – Theory of Stabbing says: “Where evil comes together, its qi must be deficient.”
9. Western medical treatment of MDS?
(1) Supportive therapy: When the patient is obviously anemic or has heart or lung disease, red blood cell transfusion can be given; RA and RA-S often have increased iron load due to repeated blood transfusion. In the presence of bleeding and infection, platelets may be transfused and antibiotics applied. Prophylactic transfusion of granulocytes and platelets has no clear efficacy in patients with MDS.
(2), vitamin therapy:Some RA-S are effective for vitamin B6 therapy, 200-500mg/day IV, which can increase reticulocytes and decrease transfusion.
(3), adrenocorticotropic hormone: about 10-15% of MDS patients, after the application of adrenocorticotropic hormone therapy, peripheral blood cell counts rose significantly, but the side effects brought about by corticotropic hormone therapy, such as easy infection and elevated blood sugar, should not be ignored.
(4) Differentiation inducers
Some cells in malignant clones of MDS patients still retain differentiation potential, and some drugs can induce differentiation of tumor cells. Currently, 1,25 dihydroxyvitamin D3, 2μg/d orally for at least 12 weeks, is commonly used. Or vitamin D 3.3 to 600,000 units intramuscularly once daily for 8 to 28 weeks. The blood picture improved in some patients during the administration of the drug. This class of drugs can cause life-threatening severe hypercalcemia, so blood calcium changes should be closely monitored. 13-cis retinoic acid has an induction of differentiation in in vitro culture, but the clinical application is not ideal, domestic mostly used all-trans retinoic acid 20mg three times daily orally. Small doses of cytarabine have differentiation-inducing effects on myeloid leukemia and have been used in MDS, especially RAEB and RAEB-T, with a remission rate of about 30% at 10-20 mg/m2/d subcutaneously for 7 to 21 days. However, the inhibitory effect of small doses of cytarabine on bone marrow still cannot be ignored, and about 15% of patient deaths are drug-related.
(5), androgens
Ethinyl testosterone (danazol) is currently the most commonly used male hormone, 600-800 mgd for 2-4 months, but there is no definite efficacy. Some reports suggest that male hormones have the possibility of accelerating the transformation to acute leukemia.
(6), Combination chemotherapy
In most cases of MDS, conventional anti-leukemia treatment is not beneficial. MDS has low tolerance to chemotherapy, poor therapeutic efficacy, and even if remission is obtained, the remission period is short. If the patient is less than 50 years old and in good clinical status for RAEB-T, conventional chemotherapy may be used as appropriate.
(7) Bone marrow transplantation
When the age is less than 50 years old and in RAEB, there is HLA homozygous donor and medical condition allows, allogeneic bone marrow transplantation can be considered.
10. Chinese medicine treatment of MDS
(1) Spleen and kidney yin deficiency: lack of color, shortness of breath, palpitations and insomnia, dry mouth and lips, little food and sluggishness, lumbar soreness and weakness, dizziness and tinnitus, hot flashes and night sweats, dry stools, red tongue with little or no moss, thin and feeble pulse.
Treatment: Nourishing the spleen and kidney.
Prescription: Radix et Rhizoma Gastrodiae, Radix Rehmanniae Praeparata, Radix et Rhizoma Lady’s Tongue, Radix et Rhizoma Drynariae, Fructus Lycii, Cornu Cervi Pantotrichum, Colla Corii Asini, Semen Cuscutae, Radix et Rhizoma Tortoise, Radix et Rhizoma He Shou Wu, Radix Astragali, Radix et Rhizoma Huang Jing, Radix Codonopsis Pilosulae, 3 grams each, Cordyceps Sinensis Powder (for dosing).
(2) Yang deficiency of spleen and kidney: dull complexion, shortness of breath, palpitation and insomnia, fatigue and drowsiness, cold form and limbs, cold pain in the waist and knees, loose stools, pale and dull tongue with teeth marks on the large side, sunken and weak pulse. The treatment
Treatment: warming the spleen and kidney.
Remedies: Radix Rehmanniae Praeparata, Radix Bupleurum, Semen Cuscutae, Antler Gum, Turtle Board Gum, Radix Astragali, Radix et Rhizoma Polygonati, Radix Codonopsis Pilosulae 10 g, Radix et Rhizoma Pilosulae, Cinnamomum Cassiae 6 g each, Cordyceps Sinensis Powder 3 g (for dosing).
11. Prevention and management of myelodysplastic syndrome?
Although the cause of MDS is unclear in some cases, many cases are due to clonal proliferation of cells caused by biological, chemical or physical factors. Therefore, preventive measures should be taken. Medical personnel should be aware of the dangers of drug abuse and be cautious in using chemotherapy drugs; radiation therapy should also strictly grasp the indications; when exposed to harmful substances such as chemicals (e.g. benzene, polyvinyl chloride) in the relevant industrial and agricultural production, labor protection should be done to prevent harmful substances from contaminating the surrounding environment in order to reduce the incidence of MDS.
(I) Life conditioning
MDS is closely related to emotions, so it is very meaningful to be emotionally optimistic and happy to prevent the disease.
(B) Diet regulation
A proper diet can maintain health, prolong life, and prevent disease. In the process of disease treatment or after treatment, through dietary conditioning can avoid the further development or recurrence of disease, and is conducive to physical recovery.
1. Pay attention to nutrition and reasonable diet, the intake of meat, eggs, fresh vegetables should be comprehensive, do not be partial food.
2. avoid chicken is Yang, moving wind, MDS mixed with the real and the virtual, the evil poison within the empty, help fire moving wind products should be avoided, especially the Yin deficiency fire, bleeding, phlegm and damp cross blocked people should pay special attention.
3. winter worm, summer grass stewed duck winter worm, summer grass nine, duck 759, ginger 3 slices, yellow wine station, water 200Inl, appropriate add salt and oil seasoning, the civil fire stew 2 hours, drink soup and eat meat. Treatment of MDS. Qi and Yin deficiency, fatigue, light red tongue, thin pulse.
(C) mental conditioning
Liver Qi stagnation, excessive thinking and the onset of MDS are closely related, and some data suggest that the onset of MDS is preceded by more than half a year of more serious mental stimulation. Therefore, it is very important to promote open-mindedness, open-mindedness, improve cultivation, maintain an optimistic attitude and cultivate a positive personality in the process of disease management.