A Dose of Haematology News #2.4.1 - Why More Black American Haemophiliacs Have Inhibitors?

I read an interesting article in the NEJM this morning. It was a study to try to explain why black population in America with Haemophilia A (FVIII deficiency) more readily forms antibody against the transfused factor VIII used as part of treatment compared to their white counterpart.

The suggested answer put forward by the team from America was surprisingly elegant and simple. There are 6 types of (wild) type, or naturally occurring FVIII in humans, conveniently named H1 to H6. Whites and majority of other races only have gene for either H1 or H2 (or deficiency of H1 or H2 in case of Haemophilia). In this study of 78 Black subjects, the haplotype of the FVIII gene was mapped and it was found that 24% of them have eitehr H3 or H4 haplotypes. Since the commercially available FVIII replacement are made from H1 and H2, those that are natively non-H1 or H2 will understandably produce antibodies against them.

In this study, the statistical analysis just about found significance at p=0.04, OR 3.6 (1.1 to 12.3). Maybe if bigger subject population was studied, the power of the study will be higher. Maybe we should do a similar study here in Malaysia to at least map the wild-type gene that our Haemophiliacs carry.

Stem Cell Treatment for Diabetics

An experimental stem cell treatment has enabled patients with type 1 diabetes to go for as long as four years without insulin injections, researchers say.

A US-Brazilian project with 23 patients found most were able to produce their own insulin after a transplant of stem cells from their own bone marrow.

The two main properties of stem cell is self-renewal (regeneration) and ability to evolve into a more specialised cell under certain circumstances. In diabetic, where the main issue is the lack of insulin-producing cells - beta cells - in the pancreas, stimulating the stem cells into beta cells is a an attractive and viable treatment option.

A Dose of Haematology News #2.1 - With Sperm Banking, Fertility is Still Preserved After Chemotherapy

CHARLOTTE, N.C., April 13 (UPI) -- The birth of a baby conceived from sperm frozen for 21 years may tie the world record for the longest-frozen, viable sperm, U.S. fertility specialists say.

Thirty-eight-year-old Chris Biblis of Charlotte, N.C., was treated for leukemia from age 13-18. In 1987, at age 16, his family encouraged him to freeze his sperm, even though there was no treatment for male infertility at the time.

Sperm banking is currently still the best way to preserve fertility in male patient after chemotherapy and bone marrow transplant. 

A Dose of Haematology News #2 - Side Effect of Donating Your Stem Cells

Even in UMMC, we do an average up to 5 mobilisation process per month. The majority are still for patient's own use (autologous mobilisation), done at the end of admiistration of chemotherapy.

In cases where the stem cells are needed for a different person, likely the sibling, the subject are given 4 days twice-daily injection of a hormone, G-CSF to promote growth of stem cell in the bone marrow. Consequently the cells will spill over into the peripheral blood and using the process of aphoresis or capturing of cells in the blood akin to dialysis.

In the recent Blood article, the American National Marrow Donor Program published their surveillance data of 2408 stem cell donors who undergo the mobilisation process. The donors are mainly for their unrelated marrow transplant program (the donors and recipient are unrelated). The side effects of exposure to G-CSF, the mobilisation process and other effect were studied.

The results showed that female subjects are more likely to develop problem with bone pain and venous access (more needing the use of invasive central catheter). Obese donor also reported more bone pain symptoms. There were also 15 patients who developed discomfort and chest pains needing hospitalisation for observation, but nothing major was established.

Full recover is the rule. The majority will have bone pain, with 1 in 4 complaining of headache and nausea during mobilisation.

A Dose of Haematology News #1 - Spinal DCE-MRI to Prognosticate AML

The article of the week that I am interested to discuss is from the National Taiwan University Hospital. It was a collaborative study from a group of Haemato-Oncologists and Radiologists. Previously, the main method of early prognostication in AML involves bone marrow examinations, either to detect early marrow reduction in disease load or the presenting cytogenetics.

The group's paper published in Blood last week highlighted their experience using dynamic contrast-enhanced MRI to detect the degree of angiogenesis in bone marrow tissue. The reduction in angiogenesis can then be translated to favourable outcome. A total of 78 subjects were recruited, ranging from all FAB subgroups of AML, including M3. The investigators perform the a baseline and a post-induction MRI on the subjects' T11 and sacral spinal bodies.

A reduction in update correlates with favourable outcome independent of age and karyotypes.