Joseph Chalhoub

Hematology lab
Ins. : Hussein Shuman

Spherocytosis

Regular red blood cells have a biconcave shape. Spherocytosis is

a blood disorder that is characterized by the production of
abnormally shaped red blood cells. When a person has
spherocytosis, the membrane that covers the red blood cells is
defective, causing the cells to be spherical in shape, instead of
biconcave. These spherical Cells are unusually delicate (fragile),
but they still are able to function within the circulatory system. It
is only when they reach the spleen that the problems begin to
develop. The spleen cannot recognize the malformed red blood
cells and therefore destroys them. This process reduces the
available red blood supply within the body and can produce a
number of worrying symptoms such as weariness, gallstones,
jaundice, arrhythmia, enlarged heart and heart failure.

recessive gene, many parents aren't even aware that they carry the defect until their children develop the
disorder. The hereditary form of this disorder is distinguished by a deficiency of spectrin. Spectrin is one of the
central proteins found on the interior of the membrane of a red blood cell. Its main function is to build and maintain
the shape of the cell's cytoskeleton. Without a sufficient supply of spectrin, the shape of the cell becomes distorted
resulting in spherocytosis.

Spherocytosis is not always inherited. Under certain Causes of Non-hereditary Spherocytosis:

circumstances, a person who is born with normal red blood cells
Acute, widespread burns
may begin to manufacture abnormal ones. For example, people
who take certain anti-malarial medications for an extended period Low blood phosphate levels
of time or who have unknowingly ingested naphthalene may
Toxins from poisonous snakes, spiders and
being to manufacture spherical red blood cells as a result of the insects
exposure to toxins. In situations such as these, spherocytosis
develops as an acquired disorder. Transfusion reaction

Traumatic injury
Temperature effect:

 Osmosis is caused by molecules

movement (water molecule), and when
temperature goes higher, molecules move
faster (kinetic energy level is proportional to
temperature). Thus with increased
temperature, osmosis is also increased, and
vice versa.
So at 37°C, osmosis is facilitated.

 Second effect of temperature is on

membrane stabiltity (in this case red blood
cell membrane). A human's phospholipid
bilayer is specially adapted for body
temperature. When we heat the phospholipid
bilayer above 37C the phospholipids move
apart, and the bilayer starts to behave more
like a fluid. This is not good: if it becomes like a fluid it can leak the cytoplasms contents.

However, when we cool the phosopholipid bilayer below 37C, the phopspholipds come closer together, and
the bilayer becomes too stiff. If it becomes too rigid, the membrane is in danger of shattering.
When comparing the osmotic fragility of RBCs at 37°C, the temperature is that of the human body, the
plasma membrane is in balance of fluidity (neither too fluid nor too stiff). In this case (at temperature of
37°C), we can compare the osmosis fragility of RBC in hereditary spherocytosis, acquired spherocytosis
and normal RBCs.

An important test which must always be carried out when spherocytes are infrequent is the osmotic fragility
test. When normal red cells are suspended in a range of hypotonic saline solutions, they do not start to lyse
until the saline concentration is reduced below 0.55g/dL. In hereditary spherocytosis, the red cells are
thicker than normal and some are already spherocytic, so that a smaller amount of fluid uptake than normal
is sufficient to burst the cells (i.e. the cells have an increased osmotic fragility). Thus, these abnormal cells
start to lyse when the saline concentration is as high as 0.6-0.8g/dL.

Usually, hemolysis occurs more in hereditary spherocytosis simply because in hereditary spherocytosis
more RBCs are affected than in non-hereditary spherocytosis (where only about 30% of red blood cells are
spherocytes, in the most severe cases).