One Life Matters :: Marrow Matters
What is Aplastic Anemia?
Aplastic Anemia is a very rare and life-threatening disease in which the bone marrow fails to produce a sufficient quantity of blood cells.
Patients with Aplastic Anemia are sometimes asked if they couldn't just eat more red meat, take iron pills, or do both to overcome their Anemia.
More specifically, the term 'Aplastic Anemia' commonly implies a condition in which the bone marrow has decreased numbers of erythrocytes, granulocytes and platelets cells circulating; the condition is called panhypoplasia. In Aplastic Anemia, panhypoplasia is simutaneously associated with leukopenia, a reduction in the number of white cells in the blood, and thrombocytopenia, a deficiency of platelets in the blood. In contrast, pure RBC aplasia, another rare disorder, involves only the red blood cell line. Aplastic Anemia is more common of the two rare disorders.
Erythrocytes are red blood cells that contain the pigment hemoglobin, which causes the red color to blood, and transports oxygen and carbon dioxide to and from the tissues. Erythrocytes are typically biconcave discs without a nucleus.
Granulocytes are white blood cells that contains numerous proteins that are responsible for helping the immune system fight off viruses and bacteria. Neutrophils, eosinophils and basophils are three types of granulocytes. Granulocytes have small particles, and form and mature in the bone marrow.
Platelets are also called thrombocytes. Platelets assist in the function of stopping bleeding by clumping and forming plugs in blood vessel injuries. A lack of platelets causes bleeding into the tissues, bruising, and slow blood clotting after injury. Platelets are colorless blood cells.
What causes Aplastic Anemia?
About half of all cases of Aplastic Anemia are idiopathic, which means they are caused by an unknown source.
When Aplastic Anemia can be linked to its cause, the more frequent causes are chemicals, such as benzene and arsenic; radiation; drugs, such as antibiotics, NSAIDs, anticonvulsants, acetazolamide, gold salts, penicillamine, quanacrine, and quainine.
The actual mechanism of how chemicals and drugs cause Aplastic Anemia is unknown; however, some studies suggest that the link may be a particular hypersensitivity, which also seems selective, perhaps genetic.
What are the Symptoms of Aplastic Anemia?
Anormocytic Anemia is defined as an anemia with a mean corpuscular volume (MCV) of 80-100 which is the normal range. However, the hematocrit and hemoglobin is decreased. Normocytic anemia is a blood problem. It means you have normal-sized red blood cells, but you have a low number of them. The presence of normal-sized red blood cells tells your doctor that you have normocytic anemia rather than another kind of anemia
Normochromic is a form of anemia in which the concentration of hemoglobin in the red blood cells is within the standard range. However, there are insufficient numbers of red blood cells. This includes: aplastic, posthemorrhagic, and hemolytic anemias and anemia of chronic disease.
Hypoplasia (from Ancient Greek πo- hypo-, "under" + plasis, "formation"; adjective form hypoplastic) is underdevelopment or incomplete development of a tissue or organ. Although the term is not always used precisely, it properly refers to an inadequate or below-normal number of cells.
A low white blood cell count (leukopenia) is a decrease in disease-fighting cells (leukocytes) in your blood. Leukopenia is almost always related to a decrease in a certain type of white blood cell (neutrophil). The definition of low white blood cell count varies from one medical practice to another. In general, for adults a count lower than 4,000 white blood cells per microliter of blood is considered a low white blood cell count. For children, that threshold varies with age. Some people who are otherwise healthy have white cell counts that are lower than what's usually considered normal, but which are normal for them.
Pancytopenia is a relatively common hematological entity. It is a striking feature of many serious and life-threatening illnesses, ranging from simple drug-induced bone marrow hypoplasia, megaloblastic anemia to fatal bone marrow aplasias and leukemias. The severity of pancytopenia and the underlying pathology determine the management and prognosis. Thus, identification of the correct cause will help in implementing appropriate therapy. (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3118050/)
Diagnosis requires demonstration of peripheral pancytopenia and the absence of cell precursors in bone marrow.
Fanconi anemia is a very rare, familial form of aplastic anemia with bone abnormalities, microcephaly, hypogonadism, and brown pigmentation of skin. It occurs in children with abnormal chromosomes. Fanconi anemia is often inapparent until some illness (especially an acute infection or inflammatory disorder) supervenes, causing peripheral cytopenias. With clearing of the supervening illness, peripheral values return to normal despite reduced marrow mass.
pure red blood cell aplasia
Pure RBC aplasia may be acute and reversible. Acute erythroblastopenia is a brief disappearance of RBC precursors from the bone marrow during various acute viral illnesses (particularly human parvovirus infection), especially in children. The anemia lasts longer than the acute infection. Chronic pure RBC aplasia has been associated with hemolytic disorders, thymomas, and autoimmune mechanisms and, less often, with drugs (eg, tranquilizers, anticonvulsants), toxins (organic phosphates), riboflavin deficiency, and chronic lymphocytic leukemia. A rare congenital form, Diamond-Blackfan anemia, usually occurs during infancy but has also been reported in adulthood. Diamond-Blackfan anemia is associated with bony abnormalities of the thumbs or digits and short stature.
HERBAL WARNING! DO NOT TAKE ANY FORM OF HERBAL MEDICATION IF YOU SUSPECT THAT YOU HAVE ANY FORM OF BONE MARROW FAILURE DISEASE. SEE MORE AT: DO-NOT-TAKE-HERBAL-MEDS.HTML HERBAL DRUGS ARE A KNOWN CAUSE OF APLASTIC ANEMIA. THEY MAY CAUSE OTHER DISEASESE.
Aplastic Anemia is a rare and life-threatening bone marrow failure disease in which the bone marrow decreases or ceases to produce blood cells. Twenty years ago, its diagnosis was a death sentence; today, bone marrow blood stem cells transplants offer hope. With proper and effective treatment, a blood stem cell transplant can replace the disease blood stem cells in the bone marrow and result in successful reproduction of blood cells. With careful medical treatment and aherence to some rigid rules for a short period of 5-6 years, and no relapses, today's Aplastic Anemia patient can be considered cured and life a long normal life.
Why "Aplastic" Anemia?
Aplastic Anemia derives its name from the process of the disease in which the blood stem cells within the bone's marrow cease to function and the cells within follicles within the marrow are replaced by fat cells. As the disease progresses, the bone marrow becomes aplastic, hence its name. Symptoms can go unnoticed until the disease has already progressed to a severe level.
Symptoms and Signs
Although onset of aplastic anemia usually is insidious, often occurring over weeks or months after exposure to a toxin, occasionally it is acute. Signs vary with the severity of the pancytopenia. Symptoms and signs of anemia (eg, pallor) usually are severe.
Severe thrombocytopenia may cause petechiae, ecchymosis, and bleeding from the gums, into the conjunctivae, or other tissues. Agranulocytosis commonly causes life-threatening infections. Splenomegaly is absent unless induced by transfusion hemosiderosis. Symptoms of pure RBC aplasia are generally milder and relate to the degree of the anemia or to the underlying disorder.
No Tests for Aplastic Anemia
There is no particular test for Aplastic Anemia; rather, it is a diagnosis derived by eliminating all other possibilities for the declining blood cell production.
bone marrow produces its destruction
Aplastic Anemia results because the bone marrow fails (to do that which it is supposed to do - create blood cells). Oddly enough, the bone marrow is responsible for creating the very cells that attack the marrow. In people with Aplastic Anemia, the bone marrow becomes diseased (see causes) and produces t-cells that trigger the immune system into attacking the bone marrow. So, Aplastic Anemia can also be classified as a type of immune system disorder or disease.
Aplastic Anemia Lacks Outword Symptoms
Aplastic Anemia can also be considered one of the silent killers, like high cholesterol, because it fails to produce highly-noticable symptoms until it has progressed to one of its more severe stages.
initial presentation of the disease
Aplastic Anemia typically presents itself with declining red blood cell counts, but that is not always the first indication.
Low b-12, red blood cells, platelets
Victoria had declining B-12 levels, then declining platelet count levels before her red blood cells ever displayed a significant decline. In the early stages of the red blood cells being affect, her red blood cells were enlarged and malformed, which could have been caused by the low B-12 level. A low B-12 level can produce malformed red blood cells, which can then be depicted on a complete blood count report (called a CBC lab) as an incorrect mean corpuscular volume (MCV) level. As well, she was pregnant at the time that the low B-12 level was discovered. Pregnancy can cause low B-12 levels.
Despite the fact that she received weekly B-12 shots, her B-12 levels didn't hold. When her general practioner saw that her blood cells were malformed, and began blasting, he referred her a hemotologist.
Typically, when there is a blood disorder effected by the bone marrow, and red blood cells are malformed, burst of immature red blood cells are produced by the marrow and injected into the blood stream in response to the body's alert to a need for more red blood cells. To complicate matters more, blasts of cells such as these can indicate a form of cancer in which the blasts are the symptom.
As well, with her being pregnant, these symptoms could have indicated HELLP Syndrome, and in fact were mistaken for HELLP by one of her doctors. HELLP Syndrome produces like symptoms with declining blood levels, but corrects itself shortly after the baby is born.
At one point, her blood specialist believed that her symptoms were produced by the pregnancy, and that they would correct after the baby was born. The declining blood cell counts held for a short period of time after the baby's birth (likely due to a termendous number of transfusions during childbirth), but again began to decline.
A segment of the medical community believes that HELLP can form into Aplastic Anemia. In Victoria's case, her Aplastic Anemia specialists do not support the theory that her pregnancy caused the Aplastic Anemia.
declining blood cell counts
Aplastic Anemia presents with declining blood cell counts. Typically, red blood cell counts decline first, but that is not always the case as noted above.
Because declining red blood cell counts rarely cause other effects initially, except for fatigue, headaches and perhaps sore joints, they can be mistaken for other causes, or even as flue-like symptoms. As the blood cell counts continue to decline, brusing may occur more easily. When the white cell counts decline dramatically, infections may start. All of these symptoms can be passed-off for other issues, including a cold or influenza.
When the platelet levels decline to nearly critical levels, dermatologic symptoms appear iwth purple dots or spots on the skin called Purpura. These spots are actually blood cells that have burst. Bruising may occur with no cause or minimal pressure on the skin.
Throughout its progression, Aplastic Anemia patients report excessive daily fatigue of a greater magnitude than can be imagined.
clinically defining aplastic anemia
Aplastic Anemia is classified by its severity: mild, severe, very severe. Severe Aplastic Anemia is defined as follows:
Bone marrow cellularity < 25%, or marrow cellularity < 50% but with < 30% residual hematopoietic cells.
Two out of three of the following (in peripheral blood): neutrophils < 0.5 x 10^9/L; platelets < 20 x 10^9/L; reticulocytes < 20 x 10^9/L. SAA diagnostic criteria may be applied to assessment at initial diagnosis or to the follow-up assessments.
aplastic anemia Information:
For more information about Aplastic Anemia, please consider these sites:
Aplastic Anemia Association
One Life Matters BLOG
Bone Marrow Donation
Bone Marrow Testing