Hematopoeitic

Question 1

Define hemostasis

Answer 1

stopping the bleeding of a damaged vessel

Question 2

What are the 4 components of blood and their functions (subcomponents)

Answer 2

Plasma - transport medium that carries everything in it, ALBUMIN makes up a majority for osmotic pressure to maintain fluid balance in vessels

Leukocytes - inflammation responders and infection control (neutrophils, eosinophils, basophils, and mast cells)

Erythrocytes - disks shaped cells that carry O2 in and CO2 out of tissues. has proteins called hemoglobin which binds to oxygen and provides the red color

Thrombocytes - platelets, clotting factors and coagulation. synthesized by kidneys, liver, and a chemokine in bone marrow.

Question 3

how is blood formed? name of the PROCESS?

Answer 3

Hematopoiesis

the kidneys produce growth factors called ERYTHROPOIETIN

in bone marrow (RED marrow) immature hemapoeitic stem cells differentiate into either myeloid or lymphoid progenitors.

MYELOID stem cell becomes an erythrocyte.

need stimulus to start making more RBC like hypoxia or blood loss, infection for WBC.

Question 4

What is the purpose of Vitamin B12 and folate in RBC development

Answer 4

DNA synthesis, without it cells may go through premature apoptosis, cant divide, or phago the precursor cells.

Question 5

In hemostasis what is the function of endothelin, nitric oxide and prostacyclin, Von willebrand Factor?

Answer 5

when vessels are injured, ENDOTHELIN released by epithelial cells makes vessels vasoconstrict.

Nitric oxide and prostacyclin levels decrease because it normally keeps platelets from adhering to the walls.

Von Willebrand factor binds to the site of injury and platelets bind to vWf. it is the glue

Question 6

what do platelets release when they become active?

Answer 6

serotonin (vasoconstrictor)

adenosine diphosphate ( activates other platelets for aggregation (clustering))

fibrinogen and calcium

Thromboxane A, vasoconstrictor and oposes prostacyclin.

Question 7

What is a platelet plug? how is it strengthened?

Answer 7

platelets bind with fibrinogen to create a plug but it is weak

fibrin strengthens

Question 8

How is fibrin formed? this process is also called?

Answer 8

Fibrinogen becomes fibrin from coagulation cascade.

THROMBIN is an enzyme from the cascade that mediates.

SECONDARY HEMOSTASIS

Question 9

Explain the extrinsic pathway of coagulation cascade

Answer 9

extrinsic meaning it was triggered by something OUTSIDE THE BLOOD

Injury occurs
A blood vessel is cut or damaged.

Tissue Factor (TF) is exposed
Cells outside the blood (like in skin or tissue) release tissue factor (Factor III) when injured.

Factor VII (7) in blood binds to TF
TF grabs onto Factor VII, which is floating in your blood, and activates it to Factor VIIa.

TF + VIIa activates Factor X (10)
This combo (TF–VIIa) quickly activates Factor X into Factor Xa.

Factor Xa leads to clot formation
Factor Xa is the key to starting the common pathway, which leads to fibrin formation (the final mesh that makes the clot).

Question 10

explain intrinsic pathway of coagulation cascade

Answer 10

The intrinsic pathway is a slower, internal clotting process triggered by blood contacting damaged surfaces, using Factors XII, XI, IX, and VIII to activate Factor X.

also activated by thrombin

Question 11

Explain the fibrinolytic system? What are the factors

Answer 11

to make sure clots aren’t forming spontaneously

t-PA (tissue plasminogen activator) and urokinase activates activates Plasminogen which becomes ACTIVE plasmin (degrading enzyme of clots)

Question 12

function of neutrophil?

neutroPHILIA

NeutroPENIA

Answer 12

first responders, most abundant. escape capillary wall go to infection site and eat microorganisms. When done, infected cells die and become yellow drainage/pus.

neutrophilia is INCREASED number of neutrophils

neutropenia is decreased nuymber of total neutrophils

prolonged infection or inflammation can cause penia.

Question 13

what organ system is most commonly affected by neutropenia

Answer 13

respiratory

(mouth ulcers, ulcers of skin, vagina, and GI tract)

Question 14

Function of eosinophils? is eosinopenia worrisome?

Answer 14

parasitic invasions, or inhalation of toxic foreign partricles

since it is already generally low in count, it is not clinically significant

Question 15

function of basophils? is basopenia?

Answer 15

hypersensitivity reactions because they have a lot of histamine to release

since it is already generally low in count, it is not clinically significant

Question 16

function of lymphocytes?

causes of increased lymphocytes?

causes of decreased?

Answer 16

immune response

increased number usually from viral infections sometimes bacterial. Leukemia, lymphoma

decreased occur with immunodeficiency syndromes, AIDS or chemo/radiation therapy.

Question 17

what is infection mononucleosis caused by?

Answer 17

infection of B lymphocytes most with EBV,Epstein -barr virus

• herpes virus
• usually targets pharynx first, sore throat

Question 18

What are lymphomas? 2 major types?

Answer 18

cancer from malignant lymphocytes. Tumors of lymphoid tissue i.e. thymus and bone marrow or secondary tissue (lymph nodes, spleen, tonsils, intestinal lymphoid tissue)

Hodgkin lymphoma

Non-Hodgkin lymphoma (NHL)

Question 19

Hodgkin lymphoma differential

where does it most often arise

two types of HL

Answer 19

most often arises in the lymph nodes of the upper body neck chest upper arms

Reed sternberg cells are abnormal type B lymphocytes that are larger but do not look nor function like it. malignant B cell that became BInucleic

Classical HL - reed sternberg, EBV infection

Nodular lymphocyte predominnant HL - cells called lymphohistiocytic cells. same B cell features and function. grtown more slowly. Males more than females

Question 20

Clinical manifestations of Hodgkin lymphoma

Answer 20

painless enlarged lymphnode on the neck or supraclavicular area - aka lymphadenopathy

MEDIASTINAL mass on chest xray, you cant palpate it nor feel it

Question 21

What are B syptoms

Answer 21

symptoms related to lymphoma.
fevers, night swearts, weight loss >10% over 6 months. can occur over weeks to months

Question 22

Stage 1-4 of Hodgkin Lymphoma

Answer 22

I. lymphoma cells found in one lymph node group i.e. neck or axillae. above or UPPER chest
II. in two lymph node groups of the same side. above or UPPER chest.
III. in lymph node above and below the diaphragm. can be in one part of tissue or an organ i.e liver lung bone
IV. several parts of one or more organs or tissues . or in an organ and in distant lymph nodes

dx: CT scan, positron emission tomography scan. MRI, bone marrow biopsy

Question 23

define refractory or reccurent/relapse

Answer 23

disease doesnt respond to treatment before or after recurrence

recurrent - disease returns after treatment

Question 24

what makes Non-hodgkin lymphoma different from HL?

Answer 24

any lymphocyte cancer with no reed sternberg cells, can randomly spread to other nodes or organs

can be aggressive or slow growing.

Question 25

What is Burkitt lymphoma? most linked to? Clinical presentations?

Answer 25

NHL, linked to EBV abdominal mass, pain, nausea RAPID growing head and neck lymphadenopathy

Question 26

what is Lymphoblastic lymphoma? Origin? clinical presentations?

Answer 26

NHL origin - precursor B or precursor T lymphocytes T-cell: thymus; retrosternal or anterior tracheal mass with difficulty breathing, chest pain B cell: painless lymphadenopathy in the neck both: B symptoms

Question 27

what is Large cell lymphoma? Origin? clinical presentations/ differential?

Answer 27

NHL origin - mature T cells, Anaplastic or mature B cell for diffuse (spread of ) large B cell Mass anywhere in the body Diffuse large b cell: mediastinal mass or neck/abdomen lymph nodes Anaplastic: painless lymphadenopathy, fever, malaise

Question 28

What are some diagnostic test of Hodgkin Lymphoma? Tx?

Answer 28

dx: tumor markers, lumbar puncture, immunoglobulin studies, serology tests of HIV and hepatitis tx: chemotherapy, radiation, immunotherapy

Question 29

What is Multiple myeloma? what does it affect? what can these cells produce?

Answer 29

Cancer of Plasma cells; since plasma cells come from B cells its a "type" of NHL. in the bone marrow multiple malignant tumors of plasma cells throughout the skeletal system they make abnormal immunoglobulins called M proteins ⦁ The myeloma cell may also secrete free antibody light chain that is excreted in the urine (Bence Jones protein).

Question 30

Clinical manifestations of Multiple Myeloma? *CRAB*

Answer 30

C- calcium high; bone is broken down releasing calcium in blood R- Renal failure; M protein damages kidneys; proteinuria A- Anemia; decreased erythropoiesis B- bone damage; bones get weakened or fractured from lesions

Question 31

what is myelodysplastic syndrome?

Answer 31

a group term for neoplasms that affect hematopoietic stem cells. (RBC, platelets, mature granulocytes)

Question 32

Acute leukemia is characterized by what? Two main origins (stem cell lines)?

Answer 32

abnormalities of the immature leukocytes (precursor). they are also called blast cells. myeloid line - myeloblasts. RBCs, platelets, Neutrophils, eosinophils, basophils, monocytes Lymphoid line - lymphoblasts. B lymphocytes T lymphocytes, NK Natural Killer cells. 80% curable

Question 33

Define Anemia?

Answer 33

decreased production of RBC due to low resources or from destruction or blood loss.

Question 34

What are some of the reasons for iron deficiency anemia?

Answer 34

lack of iron consumption, low iron absorption, increased loss from bleeding *menstruation, gi bleed* only 10% of iron is absorbed. celiac disease, atrophic gastritis, H. Pylori affect the mucosal cells which affect absorption

Question 35

Clinical manifestation of iron deficiency anemia (microcytic)

Answer 35

cyanosis of sclera brittle nails spoon-shaped nails if in children - neurocognitive impairments, decreased attention span, auditory processing, learning difficulties

Question 36

What is Macrocytic anemia? causes?

Answer 36

your RBCs are larger than normal, but not enough of them or not functioning normally deficiency of B12 (coalbumin) and folate cell color is not affected,

Question 37

where is Folate absorbed?

Answer 37

in the small intestines; duodenum and jejunum

Question 38

define aplastic anemia?

Answer 38

critical condition characterized by a reduction or absence of all three blood cell types (pancytopenia) erythrocytes, leukocytes, and platelets

Question 39

name of anemia involving B12 malabsorption?

Answer 39

Perniciuous anemia

Question 40

Sickle cell disease inheritance pattern? what portion of blood does it effect?

Answer 40

autosomal recessive, if PT inherits one abnormal gene and one normal then they are a carrier. Hemoglobin S behaves normally when oxygen is present, but when oxygen levels drop (like during exercise, stress, or illness), it sticks together and forms long, stiff rods. The rods distort into sickle shapes, can clog small blood vessels. short survival time 45 days in the body

Question 41

differential of sickle cell ANEMIA compared to SCD?

Answer 41

anemia is MORE severe and most common form of SCD. majority of erythrocytes are abnormal.

Question 42

clinical manifestations of SCD/anemia

Answer 42

hemolysis can result in jaundice or gallstones because of excess bilirubin from RBC breakdown. fatigue, pain, palor. pain is from the obstruction of small blood vessels which may lead to ischemia nd necrosis.

Question 43

what is Thalassemia?

Answer 43

Thalassemia is a genetic blood disorder where the body doesn’t make enough hemoglobin, or the hemoglobin it makes is abnormal. abnormality of globin protein chains (2 groups) a(alpha)-thalassemia b(beta)-thalassemia

Question 44

what is polycythemia? clinical manifestations?

Answer 44

excessive number of RBCs and hemoglobin; two types primary and secondary. bone marrow produces too much; considered a neoplastic disease. (primary) any disease or circumstance that leads to decreased oxygen that triggered erythropoietin response (secondary) cm: HTN, splenomegaly, visual disturbances (migraine, transient blindness), itchiness when contact with water.

Question 45

what is Thrombocytopenia? immune thrombocytopenic purpura?

Answer 45

low platelet levels, decreased production or increased destruction. Bone marrow disorders. ITP - when the immune system destroys its out platelets; autoantibody regulated. IgG reacts to the glycoprotein of platelets.

Question 46

what is thrombotic thrombocytopenic purpura? clinical manifestations?

Answer 46

coagulation disorder resulting in microthrombi in small vessels, thrombocytopenia, and bleeding. lack of a gene that makes the enzyme for destroying von Willebrand disease multiple systems involved - renal failure/insufficiency, headache, CHF, MI.

Question 47

what is thrombocythemia?

Answer 47

characterized by a platelet count > 450,000/mm3 of blood & is symptomatic when the count exceeds 1 million/mm3, which increases the risk for intravascular clotting (thrombosis).

Question 48

what is hemophilia A and B? what factors are involved

Answer 48

increased bleeding due to deficient or abnormal clotting factors; usually inherited X-Linked recessive; more likely to affect males A- factor VIII B- factor IX

Question 49

define Von Willebrand Disease type 1 type 2 Type 3 Acquired and spontaneous

Answer 49

autosomal DOMINANT, deficit of vWF; which normally causes platelets to aggregate and adhere to the vessel wall in times of injury. THE GLUE type 1- most common, autosomal dominant, mild cases, doesn't cause spontaneous bleeding type 2 - can be autosomal dominant or recessive, the parts that make vWF are smaller and more fragile; bleeding moderate to severe type 3 autosomal recessive; rare; severe bleeding problem, vWF isn't measurable acquired is from Wilms tumor, or congenital heart disease (CANNOT be passed onto offspring) Spontaneous is from rare mutation of chromosome 12 can be passed on to offspring

Question 50

define Disseminated Intravascular coagulation? causes? Clinical manifestations?

Answer 50

coagulation nd fibrinolysis is abnormally active causing micro clots everywhere. when clotting factors get used up; results in bleeding. cause: sepsis, transfusion reactions, injury, burns CM: tissue or organ ischemia, all organs; cyanosis of fingers,