Robert’s Road to Recovery Journal

Presented by the International Society for Mannosidosis & Related Diseases.

Kathleen’s Diary: August 30, 2001

Robert’s Update BMT + 42

Today has been a day of ups and downs. On the positive side, our neighbor Jo Jo today was well enough to get dialysis. His severe graft vs. host disease has impaired his kidney functions, but he was too ill for dialysis. He is now stable enough for this procedure, so this is a good sign. On a less positive note, we have gotten very ambiguous results from Robert’s engraftment study. This study determines the source of his blood cells. The good news is that the overall composition of his blood cells remains 90 donor cells, the bad news is that nearly all of the T-cells found in is blood are Robert’s cells. The test that found the T-cell result is new, and it is difficult to interpret the results. One the one hand, all engraftment tests on Robert for the last three weeks have had a very high percentage of donor cells; on the other hand, T-cells control cellular immunity, and high levels of host T-cells usually mean the bone marrow graft is failing. T-cells are highly differentiated white blood cells that mediate the body’s immune response.

The good news is that Robert is healthy, and even if the graft fails, he will most likely continue in good health.

There are several possible scenarios and courses of action:

1) Most likely – Robert’s bone marrow is still viable and mounting an attack on the donor bone marrow. The first target of this attack was the donor T-cells, which were wiped out last weekend. Cellular destruction of T-cells released pyrogens, which caused fever and may have suppressed other white cells. As Robert’s marrow continues to recover, it will begin to attack other donor blood cells. An engraftment study early next week would indicate if this is indeed the case if a higher percentage of Robert’s cells were observed. An experimental test on T-cell precursor stem cells will be performed as well. If these stem cells are Robert’s, this is the most likely diagnosis. What does this mean and what are the next steps? Robert’s bone marrow will continue to develop and reject the donor’s bone marrow. We will need to wait some time (3-6 months) until Robert is healthy enough to try a second marrow transplant. We will need to use a different bone-marrow donor, as Robert will have antibodies against the current donor.

2) Possible – Robert had T-cells in some of his organs (spleen, lymph glands, or thymus) that were immature during chemotherapy, and survived. These immature T-cells matured and became targeted on donor T-cells and eliminated them. This was the cause of fever and lowered white count last week. However, the marrow cells are predominantly donor cells, and the test on T-cell stem cell precursors will show this. What does this mean and what are the next steps? The engraftment is still in good shape. Additional immuno-suppression will be needed (drugs) to reduce host vs. graph disease. The end result would be a successful transplant.

3) Least likely – Robert’s old T-cells survived chemotherapy and are still around. Because they are highly differentiated, they will not attack donor cells. In this case, the fever and WBC count drop that occurred last weekend was caused by infection or drug effects. The stem cell test will show donor T-cell precursors, and no action is required; the graft will be successful.

It will be at least a week before we know which of these scenarios is actually happening. In any case, Robert is feeling good, and he is in no immediate danger with any of the above scenarios.

Keep us in your thoughts and prayers.

Thank you, Mark