Marrow Matters

What is Aplastic Anemia?

Aplastic Anemia is a very rare life-threatening disease in which the bone marrow ceases to produce a sufficient quantity of blood cells (red blood cells, white blood cells and platelets). About 900 new cases of Aplastic Anemia are diagnosed in the United States each year. Aplastic Anemia is not cancer. The annual incidence of Aplastic Anemia is about 2 cases per million population. [Aplastic Anemia at Marrow.org] Comparatively, an estimated 245,225 people are diagnosed with Leukemia each year in the United States.

Bone Marrow Failure Disease

Aplastic Anemia is a disease in which the bone marrow fails. It is believed to be an autoimmune disease, because the autoimmune system (or more specifically, a particular type of white blood cell) attacks the bone marrow killing blood stem cells.

Blood stem cells are seated in the marrow inside of our bones. Blood stem cells produce all of the body's blood cells. As the blood stem cells are killed off, less and less blood cells are produced and the person's blood counts drop. If a person's blood counts fall too low, transfusions may be required. Low blood cell counts can cause infection, organ failure and death.

Aplastic Anemia isn't Cancer

While Aplastic Anemia is not a cancer, it is often confused with cancer because of its symptoms, treatment or the fact that Aplastic Anemia patients are hospitalized and attend clinic at the same places as cancer patients. But it isn't cancer.

Aplastic Anemia & Leukemia Similarities

Aplastic Anemia & Leukemia (the disease with which Aplastic Anemia is most often confused) have some similarities beyond those mentioned above.

• Both Aplastic Anemia and Leukemia are life-threatening diseases.
• Both Aplastic Anemia and Leukemia will result in death if left untreated.
• Both Aplastic Anemia and Leukemia are treated with chemotherapy.
• Both Aplastic Anemia and Leukemia can go into remission and relapse.
• Both Aplastic Anemia and Leukemia can present with like symptoms: fatigue, excessive bruising, weakness, reduced exercise tolerance, weight loss, bone or joint pain, infection, fever, abnormal bleeding, and malaise, but both can also exist undetected.
• Both Aplastic Anemia and Leukemia can look alike in blood morphology and staining tests - a common issue exists with cell shape, such as anisocytosis (excess variations in sizes of red blood cells).
• Both Aplastic Anemia and Leukemia tend to produce blast cells - the body is a miraculous machine that is able to detect when an insufficient supply of blood cells exists, so it produces more; generally speaking, blast cells are immature cells that are released into the blood stream to answer the call for more cells.

Aplastic Anemia & Leukemia Differences

• Aplastic Anemia is not a cancer; Leukemia is a cancer.
• Aplastic Anemia has three types: acquired (due to an environmental factor), inherited (from family), and undetermined origin (the most common); Leukemia is usually not acquired (although some studies indicate that fetuses can acquire the disease, but it also is not inherited).
• Aplastic Anemia will fail to produce leukemia cells; leukemia produces leukemia cells. Because of all of the similarities between the various cancers and Aplastic Anemia, AA is difficult to diagnose. It isn't until the blood can be analyzed for various factors that leukemia can be identified, as well as the type of leukemia. Cytogenics is the most common way by which leukemia is excluded from the diagnostic process.

Classification of Aplastic Anemia

The severity of Aplastic Anemia is categorized as: mild, severe or very severe, depending upon the downward trajectory of the patient's blood count levels. The downward trajectory is caused by the failure of the bone marrow to produce cells as quickly as the cells die off in the body due to many factors, such as normal rates of cell death, exercise that results in faster cellular death, and bruising and bumps that kill off cells that are not replaced.

Mild Aplastic Anemia

Mild Aplastic Anemia has been known to correct itself.

There exists some debate in the medical community over whether cases that appear to be Mild Aplastic Anemia are in fact HELLP syndrome, a condition that arises in pregnant women resulting in decreased blood counts during pregnancy, but correcting itself after pregnancy.

Severe Aplastic Anemia

Severe Aplastic Anemia usually does not correct itself. It may be treated by Immunosuppression Therapy, which suppresses the T- cells that cause Aplastic Anemia. In most cases, repeated treatments or ongoing treatments are necessary, as are transfusions. The ongoing treatments cause several issues such as copies of many antibodies being introduced into the person that subsequently cause issues and may cause bone marrow transplant failure if that route is opted for in the future.

Bone marrow transplants are considered to be the only cure for Aplastic Anemia.

Sometimes, people opt not to receive a bone marrow transplant for various reasons, but most people treated with transfusions and immunosuppressant drugs do not have donor matches. You can be the match! Register at Marrow.org.

Very Severe Aplastic Anemia

Very Severe Aplastic Anemia is so severe a case of this rare disease that a bone marrow transplant is usually the only option.

Bone marrow transplants are also considered to be the only 'cure' for VSAA.

Bone Marrow Transplant

Many medical factors must be considered to develop the proper treatment plan. In cases in which the patient is young and otherwise healthy, bone marrow transplants are often highly recommended.

Bone contains marrow. Marrow is comprised of blood stem cells in a sponge-like material, as well as blood cells (neophytes, leukocytes, etc.). Although the terminology used refers to the process as a bone marrow transplant, not all such transplants contain all of the marrow; in fact, most do not.

Bone marrow transplants (BMT's) for Aplastic Anemia patients usually only include the blood stem cells. Once marrow is harvested from the donor, the blood stem cells that are removed from the marrow are used for the transplant.

Bone Marrow Transplants at Marrow.org.

Bone Marrow Donor

Finding a suitable donor is often difficult. Siblings have a 25% chance of matching. A male sibling is the ideal donor because a male's marrow is less likely to result in the recipient rejecting the donated marrow or developing "graph-versus-host" disease. Graph-versus-host disease can range from mild GVHD to very severe GVHD resulting in debilitation, and in some fewer cases can be fatal.

BE THE MATCH is a campaign being run by the National Marrow Donor Program. That program maintains a worldwide registry of potential donors for people who need a bone marrow transplant. The organization also handles donated cord blood.

Symptoms of Aplastic Anemia & Diagnosis

Aplastic Anemia could be called a silent killer, because it can realistically exist without presenting obvious external symptoms until the disease has progressed to Very Severe Aplastic Anemia.

The most common symptom of Aplastic Anemia might be the tremendous overwhelming exhaustion felt by the inflicted person; a tiredness beyond all fatigue.

In the very late stages of the disease, purple dots form on the skin; those are blood vessels breaking beneath the skin surface due to a very low platelet count.

There are other symptoms, too, but they can often be misdiagnosed. Those might include repeated miscarriages, constant headaches, changes in vision and repeated infections.

It is because of the other symptoms that Aplastic Anemia is usually detected initially, because the patient goes to the doctor, and a complete blood panel is run evidencing low blood count levels.

Diagnosis can only begin with a complete blood panel work up, which shows low blood count levels. But there is no test for Aplastic Anemia; diagnosis is a process of elimination of other possible diseases.

Causes of Aplastic Anemia

The majority of the medical community agree that the actual cause of Aplastic Anemia is unknown. Inherited Aplastic Anemia is usually determinable quite quickly, Acquired Aplastic Anemia however can sometimes be traced due to the chemical change caused by the catalyst, but the majority of Aplastic Anemia cases are determined to have been acquired from an undetermined source.

Post-Transplant Engraftment

Once the transplant has occurred, the post-transplant processes of managing the complications of a transplant begin. After the blood stem cells from a donor have been transplanted into a recipient, the waiting game begins for engraftment.

Hospital stay and release post-transplant

Most patients remain in the hospital for about 30-45 days after their transplant, depending upon many factors including engraftment and blood count levels.

If the patient's home is a distance away from the Bone Marrow Transplant Unit, the patient will likely be asked to remain in the local area (within 5 miles) of the hospital so that any life-threatening conditions can be immediately treated, and to attend clinic (2-5 times per week).

Going home will be different! Regardless of how clean you kept your home before the transplant, your new immune system is more fragile than that of a newborn baby. Newborns have their mother's antibodies to protect them until they are immunized; a BMT patient doesn't have those antibodies, and cannot be immunized for at least one year post transplant. Consequently, special considerations must be taken for household care after the transplant of an Aplastic Anemia patient including sterilizing surfaces and discarding food that is 48 hours old, or drinks that have been open for more than an hour.

Food care will be critical. A bone marrow transplant patient cannot peel a banana initially, and while he or she can eat a banana, lettuce is out of the question. Food high in Vitamin K, which is a natural blood thinner, might be forbidden in the early months, and fast food will be forbidden for the first few years at minimum. Your new immune system is fragile and new; it cannot fight off the viruses, bacteria and fungus found on fresh vegetables or fast food.

Life Changes

Many life changes will need to be made to normal routines. But these changes are small in comparison to the change that has taken place within a bone marrow transplant patient's body. Life changes can be overwhelming. Be sure to take care of mind, body and soul during this time.

Post-transplant Complications

It is critically important for all BMT patients to attend their scheduled clinics. There may be times when you just want to postpone it for a week, but it may just that appointment that would uncover an active CMV virus, or a newly contract RSV virus; the difference of a week can be life-threatening.

By following the ways to Avoid & Prevent Infections, you will well on your way to doing all you can to protect yourself.

One of the most frequent complications after a transplant is the development of Acute Graft-versus-Host Disease, and/or Chronic Graft-versus-Host Disease. An estimated 30-50% of all BMT patients develop GVHD, according to the Blood Journal; other researchers report higher numbers, estimating that 90% of all BMT patients develop some form of GVHD post transplant.

One of the common mild (Level 1) forms of GVHD involve the derma (skin). GVHD of the skin is considered to be a very common form of GVHD. The various BMT treatment centers prescribed alternative medical treatment for GVHD. Froedtert Hospital in Milwaukee ascribes to the methodologies of the Seattle Cancer Center, a leader in treating GVHD. Those treatment regimes include Photopheresis.

An ounce of prevention is worth a pound of cure

That saying couldn't apply more than it does to Aplastic Anemia BMT patients. Avoidance and Prevention to Exposure is key to long term success.

Flu & Cold Seasons Means More RSV

RSV affects nearly all school-aged children. For those with healthy immune systems, it just another cold, albeit a very bad one. But those with compromised immune systems, it can be life-threatening. Hospital is required for treatment of RSV. If you get a cold, call your doctor immediately.

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