Bubble boy disease refers to one of several genetic disorders that manifest as an inability by the body to produce T cells and B cells that battle infection or illness. Someone afflicted with this type of disorder has an immune system which functions so poorly it is typically considered to be effectively absent.

Someone with bubble boy disease does not ultimately die from the disease itself. Much like auto immunodeficiency syndrome (AIDS), a secondary infection or illness will ultimately cause the person’s death. With modern medical treatment, however, bubble boy disease can be fought and there is a decent chance of recovery through gene therapy, stem cell treatments, or bone marrow transplant.

The term “bubble boy disease” stems primarily from cases of the disorder in which the person afflicted with the illness was forced to live in a plastic bubble to avoid germs and viruses. A boy named David Vetter was the first “bubble boy” and was the person for whom the term was coined. He was born with the genetic disorder in 1971 and spent nearly the entirety of his life within a series of rooms separated from others by sheets of plastic. Unfortunately, he died in 1984 after a bone marrow transplant in which a dormant virus, which could not be found using screening practices of the time, was introduced into his system and spread like cancer throughout his body.

Rh Factors

Scientists sometimes study Rhesus monkeys to learn more about the human anatomy because there are certain similarities between the two species. While studying Rhesus monkeys, a certain blood protein was discovered. This protein is also present in the blood of some people. Other people, however, do not have the protein. The presence of the protein, or lack of it, is referred to as the Rh (for Rhesus) factor.

If your blood does contain the protein, your blood is said to be Rh positive (Rh+). If your blood does not contain the protein, your blood is said to be Rh negative (Rh-).

This Rh factor is connected to your blood type. For example, your blood may be AB+ which means that you have type AB blood with a positive Rh factor. Or, you might have O- blood which means that you have type O blood with a negative Rh factor.

It is particularly important for expectant mothers to know their blood’s Rh factor. Occasionally, a baby will inherit an Rh positive blood type from its father while the mother has an Rh negative blood type. The baby’s life could be in great danger if the mother’s Rh negative blood attacks the baby’s Rh positive blood. If this happens, an exchange transfusion may save the baby’s life. The baby’s blood can be exchanged for new blood that matches the mother’s.

Blood Types

There are four major blood types: A, B, AB, and 0. The blood types are determined by proteins called antigens (also called agglutinogens) on the surface of the RBC.

There are two antigens, A and B. If you have the A antigen on the RBC, then you have type A blood. When B antigen is present, you have type B blood. When both A and B antigens are present, you have type AB blood. When neither are present, you have type O blood.

When an antigen is present on the RBC, then the opposite antibody (also called agglutinin) is present in the plasma. For instance, type A blood has anti-type-B antibodies. Type B blood has anti-type-A antibodies. Type AB blood has no antibodies in the plasma, and type O blood has both anti-type-A and anti-type-B antibodies in the plasma. These antibodies are not present at birth but are formed spontaneously during infancy and last throughout life.

To make everything easier, this is how donations work (this without considering the Rh factor):

• A can donate to A, AB
• B can donate to B, AB
• 0 can donate to 0, A, B, AB
• AB can only donate to AB.

Lymphocytes are complex cells that direct the body’s immune system. T lymphocytes start in the bone marrow from pluripotent hematopoietic stem cells, then travel to and mature in the thymus gland. The thymus is located in the chest between the heart and sternum (breastbone). B lymphocytes mature in the bone marrow.

T lymphocytes (T cells) are responsible for cell-mediated immunity. B lymphocytes are responsible for humoral immunity (antibody production). Seventy-five percent of lymphocytes are T cells. Lymphocytes are different from the other WBCs because they can recognize and have a memory of invading bacteria and viruses. Lymphocytes continually pass back and forth between lymph tissue, lymph fluid and blood. When they are present in the blood, they stay for several hours. Lymphocytes can live for weeks, months or years.

Platelets (thrombocytes) help blood to clot by forming something called a platelet plug. There are approximately 150,000 to 400,000 platelets in each microliter of blood (average is 250,000). Platelets are formed in the bone marrow from very large cells called megakaryocytes, which break up into fragments - these cellular fragments are platelets. They do not have a nucleus and do not reproduce. Instead, megakaryocytes produce more platelets when necessary. Platelets generally last for an average of 10 days.

White blood cells (WBCs), or leukocytes, are a part of the immune system and help our bodies fight infection. They circulate in the blood so that they can be transported to an area where an infection has developed. In a normal adult body there are 4,000 to 10,000 (average 7,000) WBCs per microliter of blood. When the number of WBCs in your blood increases, this is a sign of an infection somewhere in your body. Tere are the six main types of WBCs: Neutrophils, Eosinophils, Basophils, Bands, Monocytes, Lymphocytes.

• Neutrophils are the one of the body’s main defenses against bacteria. They kill bacteria by actually ingesting them (this is called phagocytosis). They can phagocytize five to 20 bacteria in their lifetime. 
• Bands are immature neutrophils that are seen in the blood. When a bacterial infection is present, an increase of neutrophils and bands are seen.
• Eosinophils kill parasites and have a role in allergic reactions. 
• Basophils are not well understood, but they function in allergic reactions. They release histamine (which causes blood vessels to leak and attracts WBCs) and heparin (which prevents clotting in the infected area so that the WBCs can reach the bacteria).
• Monocytes enter the tissue, where they become larger and turn into macrophages. There they can phagocytize bacteria (up to 100 in their lifetime) throughout the body. These cells also destroy old, damaged and dead cells in the body. Macrophages are found in the liver, spleen, lungs, lymph nodes, skin and intestine. The system of macrophages scattered throughout the body is called the reticuloendothelial system. 

Neutrophils and monocytes use several mechanisms to get to and kill invading organisms. They can squeeze through openings in blood vessels by a process called diapedesis. They move around using ameboid motion. They are attracted to certain chemicals produced by the immune system or by bacteria and migrate toward areas of higher concentrations of these chemicals. This is called chemotaxis. They kill bacteria by a process called phagocytosis, in which they completely surround the bacteria and digest them with digestive enzymes.

Red Blood Cells (RBCs) are by far the most abundant cells in the blood. RBCs give blood its characteristic red color. In men, there are an average of 5,200,000 RBCs per cubic millimeter (microliter), and in women there are an average of 4,600,000 RBCs per cubic millimeter. 

There are several things about RBCs that make them unusual:

•  An RBC has a strange shape — a biconcave disc that is round and flat, sort of like a shallow bowl.
• An RBC has no nucleus. The nucleus is extruded from the cell as it matures.
• An RBC can change shape to an amazing extent, without breaking, as it squeezes single file through the capillaries. (Capillaries are minute blood vessels through which oxygen, nutrients and waste products are exchanged throughout the body.)
• An RBC contains hemoglobin, a molecule specially designed to hold oxygen and carry it to cells that need it. (The normal concentration of hemoglobin in blood is 15.5 grams per deciliter of blood in men, and 14 grams per deciliter of blood in women)
• The primary function of red blood cells is to transport oxygen from the lungs to the cells of the body.  
• Besides carrying oxygen to the cells of the body, the RBCs help to remove carbon dioxide (CO2) from the body. 

Plasma is a clear, yellowish fluid and can sometimes appear milky after a very fatty meal or when people have a high level of lipids in their blood. Plasma is 90-percent water. The other 10 percent dissolved in plasma is essential for life. These dissolved substances are circulated throughout the body and diffuse into tissues and cells where they are needed.

Proteins make up a large part of the 10 percent of material dissolved in plasma and tend to attract water to keep their relative concentration in blood vessels more in line with fluid outside the blood vessels. This is one of the ways the body maintains a constant volume of blood.

Plasma contains 6.5 to 8 grams of protein per deciliter of blood. The main proteins in plasma are albumin (60 percent), globulins (alpha-1, alpha-2, beta, and gamma globulins (immunoglobulins), and clotting proteins (especially fibrinogen). These proteins function to maintain oncotic pressure (especially albumin) and transport substances such as lipids, hormones, medications, vitamins, and other nutrients. These proteins are also part of the immune system (immunoglobulins), help blood to clot (clotting factors), maintain pH balance, and are enzymes involved in chemical reactions throughout the body.

Electrolytes are another large category of substances dissolved in plasma. They include: Sodium (Na+), Potassium (K+), Chloride (Cl-), Bicarbonate (HCO3-), Calcium (Ca+2), Magnesium (Mg+2). These chemicals are absolutely essential in many bodily functions including fluid balance, nerve conduction, muscle contraction (including the heart), blood clotting and pH balance. 

Other materials dissolved in plasma are carbohydrates (glucose), cholesterol, hormones and vitamins. Cholesterol is normally transported attached to lipoproteins such as low-density lipoproteins (LDLs) and high-density lipoproteins (HDLs).

Blood is a mixture of two components: cells and plasma. The heart pumps blood through the arteries, capillaries and veins to provide oxygen and nutrients to every cell of the body; the blood also carries away waste products.

The adult human body contains approximately 5 liters of blood; it makes up 7 to 8 percent of a person’s body weight. Approximately 2.75 to 3 liters of blood is plasma and the rest is the cellular portion.

Plasma is the liquid portion of the blood. Blood cells like red blood cells float in the plasma. Also dissolved in plasma are electrolytes, nutrients and vitamins (absorbed from the intestines or produced by the body), hormones, clotting factors, and proteins such as albumin and immunoglobulins (antibodies to fight infection). Plasma distributes the substances it contains as it circulates throughout the body.

The cellular portion of blood contains red blood cells (RBCs), white blood cells (WBCs) and platelets. The RBCs carry oxygen from the lungs; the WBCs help to fight infection; and platelets are parts of cells that the body uses for clotting. All blood cells are produced in the bone marrow.

But everybody’s blood is not the same. There are four different blood types. Plus, your blood has Rh factors which make it even more unique. Blood received through a transfusion must match your own. Patients who are scheduled to have major surgery make autologous blood donations (donations of their own blood) so that they have a perfect match.

Waldenström’s macroglobulinemia – A Rare Blood Cancer That Can Lead To Strokes

Waldesntrom’s macroglobulinemia (WM) is a type of cancer in the blood. Our blood contains many types of cells, one of them being white blood cells. They belong to our immune system and are meant to fight infections. Like there are many types of cells in the blood, there also several types of white blood cells (five types, actually). One of these types is lymphocytes. To make things even more complicated, lymphocytes themselves are divided into T cells and B cells (and that’s where the complication stops).

WM is a disease of B cell lymphocytes. B cells produce molecules called antibodies. Think of them as the weapons used by these cells to kill the bad guys. There is a type of antibody called IGM, which looks something like this:

• In WM B cells create too much of this antibody. The blood is then filled with these IGMs floating about. Because of their large structure, they cause the blood to become less liquid and more sticky, or viscous. The condition is then called hyperviscosity syndrome. This can lead to things such as nosebleed, dizziness, gum bleeding and blurred vision.
• In WM, like in other cancers, B cells multiply uncontrollably. They start infiltrating organs in the body. 

Symptoms can include: weakness, fatigue, bleeding from the nose or gums, weight loss and bruises in the skin. When the condition is more severe (meaning the blood is thicker) other things which may occur include: blurring or loss of vision, neurological problems (headaches, dizziness, and vertigo) and sometimes a stroke or coma may also ensue.

There’s no cure for WM. If someone doesn’t have any symptoms, usually no treatment is needed. If symptoms are present, though, usually chemotherapy is used.