consult a physician before making any medical decision in your own health care.
Hi guys, here's a synopsis of Dr. Alter's overhead we were given at camp. We didn't get a talk on this as Dr.
Alter's schedule changed and she wasn't able to give the talk. But we got the overheads, and then Dr. Lipton
gave a talk about blood production. Dr. Lipton's stuff was drawn on the white board, which doesn't translate
well to text. So if anyone has stuff that I didn't take down, please pass it along and I'll include it. If the text is
in brackets { } then it's my comment and not what I took down from the slides/doctor.
I'll work on the genetics next and post that when I get it done.
-Lesley
Synopsis of Dr. Blanche Alter's slides on Hematology 101 at DBA Camp, July 2006
[note that we didn't get a talk on this, just her overheads. I'm not including
all of them]
a.. Normal Peripheral Blood contains red blood cells, platelets, and white blood cells.
b.. Red cells contain a red pigment, hemoglobin. They carry oxygen from the lungs to other tissues that need
it. They normally live 4 months.
c.. Reticulocytes are red cells newly released from bone marrow.
d.. Platelets help blood clot. Live 7-10 days. Low numbers can lead to bruising, petechiae (tiny red dots),
nosebleeds, internal bleeding.
e.. White Cells are called Leukocytes, and their types are: Phagocytes (eaters) and Lymphocytes. Types of
Phagocytes are neutrophils, monocytes, eosinophils, basophils. They all live just hours, except monocytes
which live for days.
f.. White cell functions: nuetrophils eat bacteria and fungus (low counts can lead to susceptibility to bacterial
and fungal infections). Lymphocytes direct the other cells and make antibodies. Monocytes eat particles
coated with antibody. Eosinophils take care of allergies and fight parasites. Basophils are
related to allergies.
g.. The CBC machine draws blood from a tube into an electronic counter. The result is the CBC (complete
blood count). Normal levels for Hemoglobin (Hb, Hgb) are 12-15 grams/100ml (g/dl) for adults, lower for
children. For Hematocrit (Hct) the normal level is 35%-45%. Anemia is a low Hb/Hct.
h.. Platelet count is normally 150,000 to 400,000/ul. Thrombocytopenia is a low platelet count.
i.. White blood cell (WBC) normal count is 5000-10,000/ul. WBC differential is the % nuetrophoils, bands,
lymphocytes, monocytes, etc. in the count. Leukopenia is a low WBC.
j.. Absolute Neutrophil Count (ANC) equals the WBC times the % Neutrophils. Normal is above 1500/ul.
OK is above 500, low is 200-500, very low under 200. Neutropenia is low neutrophils.
k.. Causes of anemia are: decreased production (which will also have decreased reticulocytes), increased
destruction (which will have increased reticulocytes), or blood loss (also increased retic.).
l.. Aplastic anemia (AA): Pancytopenia due to decreased production, or
hypocellular bone marrow.
m.. Leukemia - malignant proliferation of immature cells
n.. Myelodysplastic syndrome (MDS) - cytopenia with hypercellular bone marrow.
o.. Hematopoiesis is the formation and development of blood cells. It takes place in the bone marrow and
involves stem cells.
p.. [Dr. Alter has several slides on MDS (see above) diagnostic criteria, but
as that isn't applicable to DBA I'm leaving it out.]
Lesley's notes on Dr. Lipton's talk on basic hematology of DBA on Monday at Camp Sunshine.
Differentiation is the process that a cell undergoes when it aquires functionality and loses potential. A stem
cell in the marrow (called "FBSC" [but I don't know what the FB is for]) becomes a progenitor cell. There are
two types of progenitor cells that Dr. Lipton drew: Lymphoid, which then becomes a functional blood cell -
either T-cell or B-cell, and a CRU-GEMM which can then differentiate further into a BFU-E (burst forming unit
erythrocyte), CFU-mega (colony forming unit mega??), CFU-GM (colony forming unit
granulocyte-macrophage). A BFU-E then becomes the red cells. The CFU-mega become the platelets. The
CFU-GM become the white cells. [Note here that Dr. Alter's slides had the following: CFU-E colony forming
unit erythroid, BFU-E, CFU-C colony forming unit in culture, and CFU-GM].
Erythropoiten (Ep.) is a hormone that influences red cell formation. If your hematocrit goes down, then the
Ep. goes up to tell your body to make more red cells. [Here I believe there was some discussion of why
supplementing DBA patients with erythropoiten (Procrit) shouldn't work. DBA patients have sufficient levels
of Ep., the problem is somewhere else along the production line. Dr. Lipton felt that if a DBA patient
responded to Procrit that might be an indication that they didn't actually have DBA but some other problem.]
Macrocystosis - this is the condition of having large red cells, and it's caused because having a high Ep. level
causes your body to try to quickly churn out red cells, thereby skipping some of the stages of cell division to
get the red cells out there. Also, high Ep. levels causes high levels of "fetal hemoglobin" (young) red cells.
The defect in all DBA folks with the RPS-19 gene is in the stem cell itself, but the defect doesn't manifest itself
until you get to the red cell production phase. Rarely, though, the platelets and white cells are also affected.
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