What Is Refractory Anemia?

Basic Information

nickname

Myelodysplastic syndrome with single-line or multiple-line morbid hematopoietic

Visiting department

Hematology

Common causes

The cause is not completely clear

Common symptoms

anemia

Causes of refractory anemia

The etiology of MDS is not completely clear and may have a similar pathogenesis to acute myeloid leukemia.

Clinical manifestations of refractory anemia

Most patients with MDS have varying degrees of anemia, such as pale, tired, weak, fatigued, and recurrent infections, which may be accompanied by mucosal bleeding, gum bleeding, or nosebleeds. Some patients have splenomegaly and female patients may have excessive menstrual blood.

Refractory Anemia

Blood and bone marrow

Accompanied by one or more lines of peripheral blood cell reduction, of which about half can occur in whole blood cell reduction. Morphology of bone marrow and peripheral blood cells showed abnormal cell development.

2. Bone marrow pathology

There were clusters of 3 to 5 or more primordial and promyelocytic cells in the para- and trabecular regions of the bone.

3. Colony culture of hematopoietic progenitor cells in vitro

The CFU-GM colony forming unit (CFU-GM) has fewer or fewer colonies and more clusters, and the cluster / colony ratio increases.

4. Immunophenotype

Flow cytometry can help detect abnormal proliferation of myeloid hematopoietic stem progenitor cells.

5. Cytogenetic testing

There are non-random chromosomal abnormalities, with -5 / 5q-, -7 / 7q-, +8, 20q ^- and -Y being the most common.

6. Molecular genetics testing

Somatic gene mutations can be detected in some patients with MDS. Detection of genetic mutations is helpful for the diagnosis and prognosis of MDS.

Diagnosis of refractory anemia

The diagnosis is mainly based on the corresponding symptoms of blood cell reduction, pathological hematopoiesis in more than two lines in the bone marrow, cytogenetic abnormalities, pathological changes, and in vitro hematopoietic progenitor cell colony culture results.

Refractory Anemia Treatment

Patients with low-risk MDS are treated with supportive therapy, hematopoietic, demethylating chemicals, and immunomodulatory drugs. Medium to high-risk MDS is treated with demethylation drugs, chemotherapy, and hematopoietic stem cell transplantation.

Supportive treatment

(1) Red blood cell transfusion can be given when the component blood transfusion and iron removal treatment are generally used when Hb <60g / L or accompanied by obvious anemia symptoms. The elderly or patients with reduced compensatory ability can relax the indication of transfusion.

(2) Patients with reduced and deficient anti-infective granulocytes should pay attention to prevention and treatment of infection.

2. Hematopoietic therapy

(1) Androgens: such as stanzol, testosterone undecanoate, and danazol.

(2) Hematopoietic growth factor: granulocyte colony-stimulating factor / granulocyte-single-cell colony-stimulating factor. It is recommended for MDS patients with neutrophil deficiency and recurrent or persistent infection. MDS in the blood transfusion-dependent low-risk group can be treated with erythropoietin ± granulocyte colony-stimulating factor.

3. Immunomodulators and immunosuppressants

(1) Immune modulator treatment : including thalidomide and lenalidomide, of which lenalidomide is a specific drug for 5q ^- syndrome.

(2) Immunosuppressive agents: Some patients are treated with immunosuppressive agents. After treatment with anti-thymoglobulin (ATG) / antilymphocyte globulin (ALG) and cyclosporine, the symptoms of anemia can be improved.

4. Demethylation drug treatment

Commonly used drugs include decitabine and azacitidine (AZA).

5. Allogeneic hematopoietic stem cell transplantation

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is currently the only possible cure for MDS.