Myelodysplastic syndrome (MDS), with kariotype del(11)q(11)
Short summary
A 74 year-old male, that was diagnosed as suffering from myelodysplastic syndrome (MDS) with kariotype del(11)q(11) by a bone marrow biopsy, following a gradual pancytopenia. The patient complains about slowly progressive weakness and being constantly tired.He was offered chemotherapy, but he declined this treatment because of fear that it would worsen his already severe weakness.
Patient's questions
1. What are the treatment option strategies for this medical situation?
2. Can you give details about new treatment options, relatively less aggressive, that are now in clinical trials in the US?
Medical Background
A 74 year-old male.
Medical history:
-Mild asthma treated with inhalations.
-BPH treated with an alpha blocker.
Following a gradual pancytopenia, with predominant thrombocytopenia, a bone marrow biopsy confirmed the diagnosis of Myelodysplastic syndrome (MDS), with kariotype del(11)q(11).
Clinically, patient's general health is good except for slowly progressive weakness and being constantly tired.
The patient currently not being treated for his disease. He was offered chemotherapy, but he declined this treatment because of fear that it would worsen his already severe weakness.
Following are two complete blood count tests from 2007 and 2009:
| 2007 | 2009 | |
| WBC (cells/mcl) | 3,200 | 3,300 |
| ABSULUTE NUETROPHIL COUNT (cells/mcl) | 2,060 | 2,260 |
| HEMOGLOBIN (gr/dl) | 12.3 | 11.8 |
| MCV (fl) | 100 | 103 |
| PLATELETS (platelets/mcl) | 63,000 | 30,000 |
Medical opinion
I reviewed the letter. No supporting documentation was provided. However, the patient appears to have myelodysplastic syndrome characterized by del (11). He has mild leukopenia and anemia and moderate thrombocytopenia. No mention is made of bleeding or infection, so I will presume that these problems have not occurred. I am a bit limited by my inability to review the pathology of the marrow, but I infer from the counts and the duration of the illness that this is not one of the more serious varients of MDS.
There is no indication for therapy at this time, since the counts are satisfactory for every-day living. There is no particular risk at their current levels, although the platelet count is low enough that a transfusion would be required for surgery. However, a series of alternaties are outlined below:
1) No therapy, just monitoring of counts.
2) If symptomatic anemia supervenes, erythopoietin injections (Procrit, Aranesp, Epogen) may help support the red blood cells. Sometimes this is given with G-CSF (Neupogen) if the neutrophils are depressed.
3) Occasional patients will respond to lenalidomide (Revlimid). This is particularly true if there is a chromosome 5 abnormality, but occasional patients with other abnormalities will respond as well.
4) Vidaza or Dacogen therapy is useful in ameliorating a transfusion requirement and preventing transformation into acute leukemia. The use of this relatively mild chemotherapy is associated with improved outcomes as described in Fenaux P, et al; International Vidaza High-Risk MDS Survival Study Group. Efficacy of azacitidine compared with that of conventional care regimens in the treatment of higher-risk myelodysplastic syndromes: a randomised, open-label, phase III study. Lancet Oncol. 2009;10(3):223-32. This study was undertaken in high risk patients, but vidaza is also effective in intermediate risk patients. In any case, if the disease starts to progress it is a good therapy.
5) Stem cell transplantation can be undertaken, although at the age of 74, the patient is older than the age cutoff set by most centers.
