Clinical Reference

Blood Disorder Information

Comprehensive clinical information authored by Dr. Suparno Chakrabarti and Dr. Mahak Agarwal.

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Myelodysplastic Syndromes (MDS)

Pediatric BMT — Dr. Suparno Chakrabarti

Myelodysplastic Syndrome (MDS) in Children

Myelodysplastic syndromes (MDS) are a heterogeneous group of disorders that can occur when the blood-forming cells in the bone marrow are damaged. This damage leads to low numbers of one or more type of blood cells. They represent 5–10% of all myeloid malignancies in children. MDS is characterized by ineffective haematopoiesis and increased cell death.

MDS can affect children as well, although the incidence is less than 5%. Anemia is the commonest manifestation of MDS in adults, but low white cell or platelet count is commoner in children.

The bone marrow is more often less cellular like Aplastic anemia and this entity is called Refractory Cytopenia of Childhood (RCC). This condition has to be differentiated from Aplastic Anemia.

Symptoms

  • Shortness of breath
  • Weakness or feeling tired.
  • Having skin that is paler than usual
  • Easy bruising or bleeding
  • Petechiae (flat, pinpoint spots under the skin caused by bleeding)
  • Fever or frequent infections

Classification

This is based on number of cell lines affected (i.e. 1, 2 or 3) and the number of blasts in the bone marrow.

  • Refractory Anemia
  • Refractory Cytopenia with Multilineage Dysplasia
  • Refractory Anemia with Ring Sideroblasts
  • Refractory Anemia with Excess Blasts

The three most important parameters determining the outcome: 1. Number of Cytopenias, 2. %age of blasts in bone marrow, 3. Abnormalities in the chromosomes. Based on these three parameters, an International Prognostic Scoring System has been developed.

The diagnosis is made by careful and diligent examination of Blood and Bone Marrow samples by an experienced Hematologist. Along with that study of chromosomes from the bone marrow cells (Cytogenetics) is needed.

The abnormalities of chromosomes are divided as GOOD or BAD: Good: normal, -Y, del5q, del20q. Bad: Monosomy i.e. deletion of one of the pair of chromosomes, mostly chromosomes 5 and 7 and other complex abnormalities.

Treatment

The Only Curative Treatment of MYELODYSPLASTIC SYNDROME (MDS) Is an Allogeneic BMT.

As the disease is mostly seen in older patients, a Reduced Intensity Conditioning is preferred.

The BMT should be done early, before the onset of life threatening infections or multiple transfusions which may lead to iron overload. Some of the patients with higher blast count or abnormal chromosomes might benefit from a short course of chemotherapy before the BMT.

Who can be a donor?

Although we prefer a matched family donor, a Half matched (Haploidentical) family donor or an unrelated donor provide excellent survival. However a HAPLOIDENTICAL DONOR, who has Natural Killer Cell mismatch with the patient, provides the best survival through its graft versus leukemia effect.

Other Treatments (for older patients not fit for BMT)

  • Red Cell and Platelet Transfusions as needed
  • Erythropoetin injections to reduce blood transfusion requirements
  • Hypomethylating agents such as Azacytidine or Decitabine which are milder forms of chemotherapy.

Adult BMT — Dr. Suparno Chakrabarti

Myelodysplastic Syndromes (MDS) in Adults

A myelodysplastic syndrome is a type of cancer in which the bone marrow does not make enough healthy blood cells and there are abnormal (blast) cells in the blood and/or bone marrow.

Myelodysplastic syndromes (MDS) are conditions that can occur when the blood-forming cells in the bone marrow are damaged. This damage leads to low numbers of one or more type of blood cells.

MDS is a spectrum of diseases which are characterised by anemia, thrombocytopenia and low white cell counts along with dysplasia (meaning abnormal looking) blood cells in both peripheral blood and bone marrow. They are early stages of leukemia. Many develop acute leukemia eventually, but most die because of bleeding or infections before development of leukemia.

This is commonest disease developing as a side-effect of treatment of other cancers with chemotherapy and radiotherapy and is called Therapy-related MDS or t-MDS.

Symptoms

Shortness of breath; Weakness or feeling tired; Skin paler than usual; Easy bruising or bleeding; Petechiae; Fever or frequent infections.

Classification

Refractory Anemia; Refractory Cytopenia with Multilineage Dysplasia; Refractory Anemia with Ring Sideroblasts; Refractory Anemia with Excess Blasts. Three key parameters: Number of Cytopenias, % of blasts, Chromosomal abnormalities (International Prognostic Scoring System).

Diagnosis by careful examination of Blood and Bone Marrow by an experienced Hematologist. Cytogenetics essential. Good chromosomes: normal, -Y, del5q, del20q. Bad: Monosomy (chromosomes 5 and 7) and complex abnormalities.

Treatment

The Only Curative Treatment of MYELODYSPLASTIC SYNDROME (MDS) Is an Allogeneic BMT. Reduced Intensity Conditioning preferred. BMT should be done early, before life-threatening infections or iron overload.

A HAPLOIDENTICAL DONOR, who has Natural Killer Cell mismatch with the patient, provides the best cure for MDS via Graft versus Leukemia (GVL) effect.

Other Treatments (for those not fit for BMT)

  • Red Cell and Platelet Transfusions as needed
  • Erythropoetin injections to reduce blood transfusion requirements
  • Hypomethylating agents such as Azacytidine or Decitabine which are milder forms of chemotherapy.

When to see us for MDS

Early BMT before iron overload or serious infections is critical for best outcomes.

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