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Multiple Myeloma (MM) and Cardiomyopathy

Short summary

51-year-old-male, complained of low back pain and has been diagnosed as having multiple myeloma (MM), light chain disease of the lambda type. His past medical history is remarkable for a cardiac disease. The expert believes it is cardiomyopathy, which can be amyloidotic (related to the MM) or hypertensive-atherosclerotic, or combined. The patient received anti-MM induction type of treatment (VTD combination), followed by high-dose cyclophosphamide (HDC) and autologous stem cell transplantation. The patient's low platelet count is of concern.

Patient's questions

1. Is the treatment correct and what is the best treatment ?

2. Investigational treatments?

3. Prognosis

4. Centers of Excellence and Experts in Italy

Medical Background

• Multiple Myeloma – Light Chain Disease – Lambda
• Amyloidosis
• Cardiomyopathy: Hypertensive ? Atherosclerotic ? Amyloidotic ?

The Patient History:
The patient, 51 year old male, complained of low back pain and has been diagnosed as having multiple myeloma (MM), light chain disease of the lambda type (late 2007).

His past medical history is remarkable for a cardiac disease characterized by a mild clinical picture, appears to be well controlled conservatively by medications. The precise diagnosis is not clear to me (if I get well from the papers – see note below this report). I believe it is cardiomyopathy, which can be amyloidotic (related to the MM) or hypertensive-atherosclerotic, or combined.

The patient received anti-MM induction type of treatment, including bortezomib (velcade), thalidomide and dexamethasone (the VTD combination x3), followed by high-dose cyclophosphamide (HDC) and tandem (double) autologous stem cell ("bone marrow") transplantation (in June and November 2008, consecutively), using high dose melphalan (MEL) for preparation. He then received intensification treatment with a similar combination of VTD (?), and now receives maintenance (?) based on thalidomide (?, if I understand well). As a supportive treatment he receives (received ?) the bisphosphonate zoledroinic acid, for his bone disease.

It is difficult for me to interpret the papers regarding the current (July 2009) situation of the patient. I will discuss his case assuming that he is doing relatively fine with no or minimal symptoms. The recent Hb count (12.2 g/dl) and WBC (6.3x109/l) are satisfactory, however the low (22x109/l ??) platelet count is of concern ! I could not detect enough information regarding the current MM status from the papers, so I will assume that a good response (how good ? complete response, CR? near complete very good partial response, VGPR ?) has been achieved.

Medical opinion

Q & A & Discussion

Is the treatment correct and what is the best treatment ? In one word the answer is yes ! The current approach to an aggressive (and this is the case) of MM in a young patient is to induce with an anti-MM therapy, and this can be achieved by a combination of 2-3 medications, and the combo used here (VTD) is one of the more known and used in clinical practice. Although other combinations, such as using only thalidomide and dexamthasone (ThalDex) and leaving the bortezomib for later disease stages, or using revlimid (lenalidomide) with or without dexamthasone (RevDex) exist – the patient received a very well accepted treatment. This step is usually followed in young adults by one or two (tandem) stem cell transplants, which was the case here. From here we are still puzzled. The question of continuing maintenance treatment (which is given to the patient, if I understand well) is still open and an unresolved issue. Obviously, the current disease status (CR ? VGPR ?) matters and this information is still lacking (to me). Nevertheless, the issue of maintenance in an attempt to prevent disease recurrence – is still controversial.

Investigational treatments: Yes, there are today many investigational protocols, most of them applying the recently approved medications (bortezomib, thalidomide, lenalidomide, doxil) in various combinations and dosing, as well as temporal regimens, and few of them introduce more novel and experimental approaches (vaccines, for example). However, before one "jumps" on an investigational treatment he has to bear in mind: 1) These treatments are usually given in academic centers (see below). 2) Not every patient is suitable for these approaches. 3) The patient has to balance between the current conservative "old" approach and the novel unknown yet: can he gain or lose ? From what I see in the papers of the patient, I fail to see why he needs to switch to an investigational protocol now (unless I miss something and the patient either suffers or the lab results regarding the disease status are poor and progressing).

Prognosis: It is difficult for me to predict the prognosis of a particular patient, especially a patient whom I have never seen and whose documents, at least those provided to me are incomplete and there is missing information (see above regarding the disease status). Unfortunately, the administration of aggressive approach, such as transplants and the introduction of novel agents has not resulted in the disease cure, but the good news is that the survival is significantly prolonged over the years and quality of life is improved. So, it is difficult to predict the particular case of this patient. It is reasonable to assume that at some point the disease may recur, but on the other hand it may take years before it happens or maybe he may join the few who benefit from treatments for years. Unfortunately, we cannot predict who will relapse and when and the best current approach is to continue to be followed (also see below).

Centers of Excellence and Experts in Italy: Yes, there are several:
o Dr. Antonio Palumbo, Torino
o Dr. Mario Boccadoro, Torino,
o Dr. Michelle Cavo, Bologna
o Dr. P. Musto, Piza

Several other points that has not been raised but I think are important:
o The low platelet count (22x109/l ?? right ?) is of concern ! If indeed, this is the case, one has to try to understand the reason for that, since the risk of serious bleeding is substantial. The two main common reasons that I can think of are medications (every drug but thalidomide is the main to be suspected !) or bone marrow damage due to heavy prior treatments. Thus, in the absence of clear cut indications for thalidomide now, I would consider discontinuing the thalidomide, considering the discontinuation of any unnecessary drug and try to raise the platelet count. Has the bone marrow examination showed anything regarding the megakaryocytes ? Also, administration of hexakapron or any other anti-fibrinolytic agent might help in reducing the risk of bleeding.

o Revlimid (lenalidomide), a relatively novel agent which is considered very effective in MM has not been administered yet to the patient. This allows us to keep on the shelf for future purposes if and when it is required.

o Other approach, such as allogeneic stem cell transplant, is reasonable in some patients, but given the risk, such treatment is preserved as the last, only if there is no alternative. However, a search for a possible donor in case that the patient needs it – makes sense.

o Conitnued follow up is very important (see above) in order to detect possible disease progression which will require treatment (revlimid ?). A very accurate lab parameter for such a follow up is the serum free light chains (FLC) which are used more today and serve as a very sensitive predictor of disease status.

o The cardiac status and the amyloidosis is indeed a matter of concern. However, in this particular case it appears to be well treated and controlled.