Patho Hematologic: System - lecture 13

What are the functions of the hematologic system?

deliver substances, remove waste, defend against pathogens and injury, maintain acid-base balance

What percent of the blood volume is plasma?

~55%

What percent of blood is formed blood?

~45%

What percent of formed blood is RBC?

~99% 

What percent of formed blood is WBC?

~<1%

What percent of formed blood is platelets?

~<1%

What part of the blood has the function of transporting nutrients, hormones, waste products, regulating BP, pH and temp, and protection by helping with antibodies and clotting?

plasma

What is the function of the plasma protein albumin in plasma?

keep oncotic pressure, fluid in compartment, transport hormones and drugs, maintain BP

What is the function of the plasma protein globulins in plasma?

help with immunity

What is the function of the plasma protein fibrinogen in plasma?

help with clotting

What is the part of the blood responsible for tissue oxygenation and removal of CO2 to keep pH in a normal range?

erthrocytes - RBCs

What is the cycle for RBCs?

100-120 days

Where in the RBCs made?

bone marrow 

What part of the blood defends against infection, foreign particles and abnormal cells, assists with tissue repair and acts in the tissue but is transported by circulation?

leukocytes - WBC

What structures classify leukocytes?

granulocytes and agranulocytes 

What is the leaving of WBCs to go to the site of injury?

diapetesis

What are examples of granulocytes?

neutrophils, basophils, and eosinophils

What is the examples of agranulocytes?

monocytes, macrophages, and lymphocytes

What part of blood is essential for blood coagulation and control of bleeding, assists with healing, and aggregates when injury occurs?

thrombocytes - platelets  

Where do platelets fragment from?

megakaryocyte

What organ is very vascular and filters the blood, removes antigens, removes old or damaged cells, produces antibodies, stores RBCs and platelets, and recycles iron?

spleen 

What does the spleen store extra of?

Blood 

What has the function to remove pathogens, damaged cells, and cancer cells, returns lymph fluid to circulation, creates antibodies, and has macrophages?

lymph nodes 

What has the function of hematopoiesis (makes blood) and immune support?

bone marrow

What part of bone marrow is in the pelvis, sternum, mandible, femur, and humerus, where the blood is made?

red (active) marrow

What part of bone marrow is in all other bones and does not create immune cells?

yellow (inactive) marrow

In babies what type of bone marrow is in all of their bones?

red marrow

What is blood cell production that occurs in bone marrow, liver and spleen in fetus and in bone marrow after birth?

hematopoiesis 

Where does hematopoiesis start?

hematopoietic stem cells

What are the types of hematopoietic stem cells?

myeloid and lymphoid

How does the body know how many cells to make?

hormone

What hormone stimulates proliferation of progenitor cells making WBC?

colony-stimulating factors (CSFs)

What hormone is produced primarily by the kidneys making RBC?

erythropoietin (EPO)

What hormone is produced in the liver stimulating platelet production?

thrombopoietin (TPO)

What is erythropoiesis?

RBC development

What begins in the bone marrow?

erythropoiesis

Where does erythropoiesis start?

progenitor cells

What are young RBCs, and is a good indicator if RBC activity?

reticulocyte

What regulates RBC production?

negative feedback loop 

What conditions cause an increase in red cell production?

hypoxia and anemia

What are nutritional requirements for erythropoiesis?

proteins, vitamins (B₁₂, B₆, B₂, E, C, folic acid, pantothenic acid and niacin), minerals (iron and copper)

What and where are old RBC destroyed?

macrophages - spleen

Where are old RBC destroyed if the spleen is dysfunctional?

liver

Where are macrophages excreted from?

liver, intestine or bile

What is oxygen-carrying protein located within the RBC, which is made of 4 chains (2 alpha and 2 beta)?

hemoglobin (Hgb)

What attaches to the end of the hemoglobin chain?

4 O2 molecules 

What is needed to form Hgb and carry oxygen?

iron

What part of total iron bound to heme?

1/3

How many mg are needed for RBC production?

25

How many mg per day is needed from dietary source?

1

Who needs more iron than others?

pregnant women 

What leads to iron overload, liver failure, damage to the heart and endocrine disorders?

unbound free iron

What is a meshwork of protein (fibrin) strands that stabilizes platelet plug?

clotting factors

What clotting factor is activated by internal damage to the vascular endothelium?

intrinsic pathway 

What clotting factor is activated by external damage of tissue?

extrinsic pathway

What leads to the activation of factor X, which leads to clot formation?

intrinsic and extrinsic pathway 

What are are pediatric heme considerations?

blood cell counts increase above adult levels at birth

Why do infants increase blood cell counts?

prepare for birth, cutting of umbilical cord, fetus produces excess EPO to stimulate extra RBCs production

What are elderly heme considerations?

erythrocytes are replaced more slowly, bone marrow production decreases

What is reduced total number of RBCs and/or Hbg in the circulating blood or in the quality?

anemias

What are symptoms of anemia?

fatigue, weakness, dyspnea, pallor

What is the physiologic manifestation of anemia?

anemias

What are anemias classified by?

size and color

What suffix means size?

-cytic

What is high mean cell volume?

macrocytic

What is low mean cell volume?

microcytic

What suffix means hemoglobin content or color?

-chromic

What on CBC do you look at to see hemoglobin content?

mean corpuscular Hgb Concentration (MCHC)

What type of anemia is when RBC in normal size and color, cell has no issues with formation of RBC but there is not enough of it?

blood loss anemia

What anemia occurs if the loss of blood is greater than the replacement capacity of bone marrow?

chronic blood loss

What is the Hgb goal?

>7

What type of anemia is caused by vitamin B12 deficiency but was once fatal?

pernicious anemia

What are the manifestations of pernicious anemia?

difficulty walking, numbness/tingling or hands and feet, smooth and beefy red tounge, neurologic manifestations

What is the most common iron deficiency that is caused by dietary deficiency, impaired absorption, increased requirement, chronic blood loss, and chronic diarrhea?

iron deficiency anemia 

What are early symptoms of iron deficiency anemia?

pale earlobes, palms, conjunctiva, fatigue, weakness, SOB

What does progression of iron deficiency cause?

koilonychia, cheilosis, stomatitis, glossitis, dysphasia

What type of anemia is when the RBC becomes small with low hemoglobin concentration and is caused by underlying causes?

anemia of chronic disease

What does anemia of chronic disease result from?

dec RBC life span, suppressed production of EPO, ineffective bone marrow response to EPO, altered iron metabolism

What are the manifestations of anemia of chronic disease?

mild, vauge

What is a blood cancer in which bone marrow makes too many RBCs, WBCs and platelets?

polycythemia vera 

What are the manifestations of polycythemia vera?

inc blood volume and viscosity, hypercoagulopathy (high risk for blood clots), fatigue, HA, dizziness, SOB, blurred vision, redness of face and hands 

What is treatment of polycythemia vera?

blood letting and low dose aspirin

What is a malignant disorder of the bone marrow and blood that causes crowded bone marrow, decreased function of stem cells, and high or low WBCs, plts, Hgb (pancytopenia)?

leukemias

What type of leukemia is present if undifferentiated or immature cells with blast cells are present with rapid and abrupt onset, making it a medical emergency?

acute leukemia

What type of leukemia is when the predominant cell is mature but does not function normally and has slow progression?

chronic leukemia 

What type of acute leukemia is too many lymphoblasts and occurs mostly in children?

acute lymphocytic leukemia (ALL)

What type of acute leukemia is characterized by too many myeloblasts and occurs mostly in adults?

acute myelogenous leukemia (AML)

What are the manifestations of acute leukemias?

fatigue, bleeding, fever, anorexia, and neurologic symptoms

How do you diagnose acute leukemias?

peripheral blood and bone marrow biopsy

What supportive treatments can be done for acute leukemia?

blood transfusions, antibiotics, antifungals, and antivirals 

What type of chronic leukemia is characterized by too many lymphocytes which is the most common adult leukemia?

chronic lymphocytic leukemia (CLL)

What type of chronic leukemia is characterized by too many myeloid cells?

chronic myelogenous leukemia (CML)

What are the symptoms of chronic leukemia?

lymphadenopathy, fatigue, night sweats, bruising/bleeding, infections, enlarged spleen 

How do you diagnose chronic leukemia?

peripheral blood and bone marrow biopsy 

What disease is characterized by enlarged lymph nodes?

lymphadenopathy (LAD)

What type of lymphadenopathy is characterized by drainage of an inflammatory lesion located near the enlarged node, such as in a local infection?

localized lymphadenopathy 

What type of lymphadenopathy occurs in the presence of infections, autoimmune diseases, or disseminated malignancy?

generalized lymphadenopathy 

What is a diverse group of blood cancers that develop from the malignant lymphocytes in the lymphoid system, and is the most common blood cancer in the US?

lymphoma 

What type of lymphoma is less aggressive and has only 4 types?

hodgkin lymphoma

What type of lymphoma is more aggressive, has over 70 types, slow growing, or aggressive?

non-hodgkin lymphoma