(ppt) Unit 6 Hematology Flashcards
Blood is made when the bone marrow receives _________ which is a hormone that is secreted by the kidney.
erythropoietin,
Each individual hemoglobin can carry _ oxygen molecules.
4
___ of the blood is water, which is why dehydration is a big cause of hypernatremia.
90%
___ is proteins, like albumin that controls the plasma colloid oncotic pressure
8%
The hematocrit is …
the volume of the RBCs divided by the total blood volume at a certain point of time.
Hemoglobin (<g/dL) normal vales for
1. Male
2. Female
3. Child
4. Infant
5. Newborn
- Male 14-18
- Female 12-16
- Child 11-16
- Infant 10-15
- Newborn 14-24
Hematocrit (<%) normal values for
1. Male
2. Female
3. Child
4. Infant
5. Newborn
- Male 42-52
- Female 37-47
- Child 31-43
- Infant 30-40
- Newborn 44-64
low hemoglobin levels indicate…
anemia
hematocrit is the quick method to diagnose…
anemia
Neutrophils are the most abundant WBC and are the ones that show up in EARLY inflammation/infection but die off in _______
1-2 days.
Eosinophils are induced by … They are elevated during ….
IgE mediated hypersensitivity reactions and to parasites.
type 1 hypersensitivity, allergic reactions & asthma.
Hematopoiesis ↑ in response to (4)….
proliferative disease, hemorrhage, hemolytic anemia (erythrocytes destroyed), chronic infection,
Iron is transported in the blood bound to….
transferrin
a glycoprotein synthesized primarily by the liver
Iron balance is maintained through …
controlled absorption NOT through excretion
Remember… not enough RBCs means not enough Oxygen, AND …
hypoxemia can lead to heart failure!!!
Microcytic-Hypochromic Anemias
Characterized by red cells that are abnormally small and contain reduced amounts of hemoglobin.
Lack of intrinsic factor can lead to…
pernicious anemia
IF is needed for…
B12 GI absorption
Lack of cobalamin (B12) can lead to…
Macrocytic (megaloblastic) anemia (B12 deficiency anemia)
B12 is needed in…
the synthesis of DNA and RNA, maturation of RBCs, and facilitates folate metabolism
Microcytic-hypochromic anemias
Characterized by red cells that are abnormally small and contain reduced amounts of hemoglobin.
What is the most common type of microcytic hypochromic anemia caused by?
Occurs during pregnancy/continuous blood loss most common causes (2-4ml/day enough to cause it).
Fe deficiency
3 stages of Fe deficient anemia are…
3 overlapping stages:
- iron stores are depleted but RBC production continues.
- insufficient amounts of iron transported to marrow and production begins to become deficient
3 hemoglobin deficient RBCs enter circulation and replace normal aging RBCs
What is Sideroblastic Anemias?
a heterogeneous group of disorders that are characterized by anemia of varying severity that occurs due to inefficient iron uptake.
What causes ringed sideroblasts in the bone marrow?
Red cells containing iron granules that haven’t synthesized into hemoglobin, instead are ringed in a circle around the nucleus
Iron gets sequestered into the tissue, so tissue levels of iron are increased.
Related to altered synthesis of RBCs in bone marrow
May have bronze-colored skin due to iron overload
How does Cu deficiency lead to sideroblastic anemia?
Copper deficiency causes reversible SA by interfering with the conversion of ferric iron to ferrous iron
Rare but can be due to gastrectomy and prolonged parenteral nutrition that does not contain copper supplements
Hypothermia causes decreased heme synthesis and decreases its incorporation into hemoglobin
Characterized by red cells that are relatively normal in size and hemoglobin content but insufficient in number
Just too few RBCs
Normocytic-Normochromic Anemias
What are the 5 distinct normocytic-normochromic anemias?
Aplastic anemia
Post hemorrhagic
Hemolytic anemia
Sickle cell
Anemia of chronic inflammation
What is aplastic anemia>?
Syndrome of bone marrow failure characterized by pancytopenia and marrow hypoplasia
Pancytopenia is… and is caused by…
Reduction or absence of all three blood cell types
Failure or suppression of bone marrow to produce adequate amounts of blood cells
Pure red cell aplasias are associated with..
autoimmune, viral, neoplastic disorders
Fanconi syndrome
Rare genetic autosomal recessive disorder accompanied with multiple congenital anomalies – bone marrow failure
Anemia often referred to as Fanconi Anemia
Erythropoietin is
hormone that tells bone marrow to make RBCs
Anemia of chronic disease (ACD) is a type of normocytic normochromic anemia characterized as… and is seen in (6)…
Mild to moderate anemia seen in: AIDS, rheumatoid arthritis, lupus erythematosus, hepatitis, chronic renal failure, and malignancies
Pathologic mechanisms of ACD (3)
Decreased erythrocyte life span
Ineffective bone marrow response to erythropoietin
Altered iron metabolism
Macrocytic-Normochromic Anemias aka characterized by… caused by…
- megaloblastic anemias (Fat RBC’s).
- bigger than usual Red blood cells. and defective DNA synthesis
- deficiencies in vitamin B12 or folate
What causes
1. B12 deficiency &
2. Pernicious Anemia
(Most common type of macrocytic anemia)
Two types of B12 deficiency anemia:
- Caused by lack of B12 in diet = AKA B12 deficiency anemia
- Caused by Vit B12 deficiency due to lack of Intrinsic Factor (IF) = AKA Pernicious Anemia
Forms of pernicious anemia (2):
Forms:
- Congenital
- d/t adult onset gastric mucosal atrophy with destruction of PARIETAL CELLS.
Gastritis, Crohn’s, or anything that destroys parietal cells
What is folate deficient anemia (type of anemia and importance of folate) ?
Macrocytic-Normochromic anemia
Folic acid needed for RNA/DNA synthesis inside the RBC
Folate absorbed from
Duodenum.
Folate deficiency occurs…
more frequently than B12 deficiency (especially in malnourished or alcoholics)
Describe HDN (hemolytic disease of the newborn.
Alloimmunity disease in which maternal blood and fetal blood are antigenically incompatible
Mom’s immune system produces antibodies against fetal RBCs
Fetal RBC recognized as foreign/defective and removed from circulation
SCD (HbSS) is…
a group of disorders that is characterized by the production of abnormal hemoglobin S within the red blood cells. 3 types
- Sickle cell anemia (most severe)
- Sickle cell-thalassemia disease
- Sickle cell-hemoglobin C disease
In SCD, sickling depends on (3) :
degree of oxygenation, pH, and dehydration
How can sickling be reversed?
Not permanent: regain shape with reoxygenation and rehydration Can lead to crisis
Very painful!!!!!
Best treatment: Hydroxyurea
2 ways that hydroxyurea works…
The first way is in the red blood cells. … With higher levels of hemoglobin F, red blood cells are less likely to sickle and cause problems. The second way that hydroxyurea works is by lowering the number of neutrophils (infection— fighting white blood cells).
Sickle cell-hb C disease _____ than sickle cell anemia
milder
Sickle cell-_______ is mildest form
thalassemia
_______ and _________are HUGE risk factors for Sickle Cell Crisis!
Illness and DEHYDRATION
Thalassemias cause impaired rate of alpha or beta adult hemoblobin sysnthesis
Referred to as… depends on….
Major or Minor
Depends on # of defective genes and mode of inheritance
Thalassemia minor can cause…
This is usually _____ to the fetus.
……… and the fetus has a grossly enlarged heart and liver.
- hydrops fetalis and fulminant intrauterine congestive heart failure.
- fatal
- Edema and massive ascites occur
Myeloproliferative Red cell disorders occur ….
when one or more marrow elements are being produced in larger quantities than usual
Most Common myeloproliferative disorders include (4):
Polycythemia Vera, Essential Thrombocythemia, Primary Myelofibrosis, and Chronic Myelogenous Leukemia (CML).
Polycythemia:
excessive red cell production. 2 forms
1. relative
2. absolute
absolute polycythemia has 2 types:
primary and secondary
primary polycythemia is aka…
polycythemia vera
polycythemia vera is…
Mutation in the Janus Kinase 2 (JAK2) gene.
Overproduction of red cells and higher level of hemoglobin—makes blood thick, so it slows the flow, and prevents O2 from getting to organs. Also increases risk of clots!
Manifestations of Polycythemia Vera
thick, sticky blood. Intense, painful itching, which is intensified by heat or exposure to water) is a unique feature of PV.
Acute leukemia
Presence of undifferentiated or immature cells, usually “blast” cells
(Increased risk in children with Trisomy 21 (Down Syndrome)
Chronic leukemia
Predominant cell is mature but does not function normally
Leukemia is …
a clonal malignant disorder of both the blood and the blood-forming organs.
There are 4 different classifications of Leukemia:
- Acute lymphocytic leukemia (ALL)
- Acute myelogenous leukemia (AML)
- Chronic myelogenous leukemia (CML)
- Chronic lymphocytic leukemia (CLL)
The common pathologic feature of all forms of leukemia is …
an uncontrolled proliferation of malignant leukocytes which causes overcrowding of the bone marrow and leads to a decreased production and function of normal hematopoietic cell
Acute leukemia is characterized by …
undifferentiated or immature cells which are typically blast cells.
Acute leukemia has a fast and abrupt onset and usually leads to a short survival time.
Chronic leukemia is characterized by …
more mature cells but these cells do not function the way they should.
The onset of the disease is gradual and tends to lead to a longer illness but a relatively longer survival time.
( for leukemia) In addition to looking at whether or not the disease is acute or chronic, we also look at the type of cell that is involved.
We characterize the disease by …
whether or not a myeloid or lymphoid cell is involved and also by looking at the degree of differentiation.
Again, in acute we usually see undifferentiated (immature cells) and in chronic we will see differentiated mature cells that don’t function properly.
Acute lymphocytic leukemia is common…
Least common overall, MOST common in kids
Genetic conditions associated with leukemia includes…
Down syndrome,
AML is caused by… most common in…
acquired oncogenic mutations that impair differentiation and mostly adults are affected
CML is caused by…
Genetic mutation of translocation that results in a fusion gene called the PHILADELPHIA CHROMOSOME.
The new gene produces an enzyme called tyrosine kinase, which promotes activity and growth of immature white blood cells (aka Myeloid cells) in the bone marrow.
which leukemia is being decribed?
adults (35% of cases are AML)—MOST common for adults
Abnormal B cells grow and spread in the blood stream where they crowd out healthy cells
Chronic lymphocytic leukemia (CLL)
Reciprocal translocation between chromosomes 9 and 22 that causes a fusion of the BCR1 gene region from chromosome 22 and the protooncogene ABL1 from chromosome 9. called the
Philly chromosome
Signs and symptoms of leukemias:
Secondary to bone marrow suppression
Secondary to overproduction of abnormal white cells that “push out” normal RBC, WBC and platelets
Tx of leukemia prognosis is…
Prognosis in children good; in adults improving but still high mortality
Lab Values:
WBC:
- normal _____
- critical values ______
Critical Values = patient is at risk for immediate harm/death!
- is 5000-10,000µL,
- <200µL or >30,000µL
Describe what shift to left and right means for WBCs
Shift to left means there are lots of immature neutrophils (aka bands) being released quickly because there is inflammation or infection somewhere.
Shift to right means extra mature neutrophils with very few bands, seen in liver disease, megaloblastic anemia, drugs, cancer.
Leukopenia is never normal, when is it very dangerous?
WBC < 1000/mm3 increases risk of infection drastically
Requires strict aseptic technique, place patient on strict isolation! Treat the infection ASAP!!!!
Neutropenia:
A reduction in neutrophils to < 2000/mm3
VERY bad! Huge risk for infection (next slide)
Neutropenic patient with fever is assumed to be…
infected and at risk of developing sepsis rapidly
assessment, cultures and antibiotics needed asap!!!
Requires STRICT asepsis!!!!!!!
Secondary Lymphoid Organs:
Spleen has…
Lots of blood vessels to store blood AND is the site of fetal hematopoiesis
What’s Mono?
Acute, self-limiting infection of B lymphocytes transmitted by saliva, respiratory, genital & rectal secretions.
Commonly caused by the Epstein-Barr virus (EBV)—85%
B cells have an EBV receptor site
Most common cause of death for Mono?
splenic rupture
monospot is…
qualitative test for heterophilic antibodies
Hodgkin’s lymphoma (HL) is…
a malignant lymphoma that progresses from one group of lymph nodes to the next and includes the development of systemic symptoms and the presence of specific B cells called “Reed-Sternberg” cells (aka RS cells).
Physical and lab findings of HL:
Physical findings
- Adenopathy (painless), mediastinal mass, splenomegaly, and abdominal mass
- Presence of Reed-Sternberg cells in the lymph nodes
- B Symptoms: Fever, weight loss, night sweats, pruritus
Laboratory findings
- Thrombocytosis, leukocytosis, eosinophilia, elevated ESR, and elevated alkaline phosphatase
the key to differentiating between Hodgkin and Non-Hodgkin…
Reed-Sternberg cells are the key to differentiating between Hodgkin and Non-Hodgkin…
Non-Hodgkin has NO Reed-Sternberg
Burkitt lymphoma is… 3 main types… :
- B cell NHL tumor with unique clinical and epidemiologic features
- 3 main types: endemic, sporadic, and immunodeficiency-related.
endemic BL usually occurs in…
Africa and is linked to the EBV. Involves rapidly growing jaw and facial bone tumors.
Describe patho of Multiple Myeloma
B cell cancer that involves proliferation of malignant plasma cells.
The malignant cells infiltrate the bone MARROW where they become PAINFUL skeletal system tumors.
In the marrow, these tumors increase the osteoclastic bone destruction, which is very painful
Bence Jones proteins develop and pass through the glomerulus of the kidney where they damage the renal tubular cells.
Ultimately, the Bence Jones proteins pass into the urine, where they aid in diagnosis.
What is platelet count?
of circulating platelets
Normal PTT
Normal Partial Thromboplastin Time is 25 to 39 seconds.
Normal PT
Normal Prothrombin Time is 11-16 secs
Normal thrombin time is…
14-19 secs
Platelets:
1. normal range
2. thrombocytopenia
3. critical values (for bleeding, for clotting)
150,000µL-400,000µL
< 140,000µL thrombocytopenia
Critical values: <20,000 µL – major risk bleeding
> 1,000,000 cells/µL major risk clotting
______ ________ causes a broad range of hemostasis disorders and involves defects in coagulation, fibrinolysis, and platelet number and function
- Factor VII (1st to decline); Facto II and X are next. Factor IX only when destruction is bad. Factor V levels are a direct reflection of liver cell decline
liver disease
thrombocytopenia Considered significant when Platelet count <100,000/mm3 (3)
<50,000/mm3—hemorrhage from minor trauma
<15,000/mm3—spontaneous bleeding without injury or trauma
<10,000/mm3—severe bleeding
_______ is the most common cause of drug-induced thrombocytopenia
Heparin
What are the 3 clotting disorder disc
THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP)
Immune Thrombocytopenic Purpura (ITP)
Disseminated Intravascular Coagulation (DIC)
THROMBOTIC THROMBOCYTOPENIC PURPURA (TTP)
Loss of ADAMTS 13 protein that processes VonWillebrand factor
Immune Thrombocytopenic Purpura (ITP)
Autoimmune with IgG antibody that targets platelet glycoproteins and sequesters and removes the platelets from the circulation
Disseminated Intravascular Coagulation (DIC)
Widespread activation of coagulation system leads to fibrin clot formation in the small/medium vessels.
Clotting is so severe that all the platelets and clotting factors are consumed, leaving the client at an increased risk of bleeding!
hemophilias
bleeding disorders
Tx of hemophilias?
Hemophilia A tx’d with recombinant factor VIII
Hemophilia B (Christmas Disease) tx’d with recombinant factor IX
