Module 2: Hematologic Disorders

1. WBC = leukocyte...fight infection

2. RBC = erythrocyte...carry oxygen

3. Platelet = thrombocyte...aid in clotting

REVIEW: What's WBC, RBC, and Platelet?

1. Penia = too few cells

2. Cytosis = too many cells

3. Anemia = too few RBCs

What's Penia, Cytosis, and Anemia?

Type of cancer of hematopoietic system with uncontrolled proliferation of leukocytes, causing overcrowding of bone marrow & decreased production of normal hematopoietic cells

What is Leukemia?

1. Leukocytosis (norm count is 6k-10k, during infection is 10-20k)

2. Thrombocytopenia --> easy bleeding & bruising

3. Anemia --> fatigue, SOB

4. Ease of infection bc WBC function impaired

What are the 4 S&S of Leukemia?

- Type of cancer that's mostly idiopathic etiologically, but may have genetic component

- Predominant cell involved in becoming cancerous is B-lymphocytes, specifically in the plasma cells

What is Multiple Myeloma?

1. Myelo = bone marrow (myo is muscle) so don't get confused

2. Even though it has JUST oma (opposed to sarcoma) THIS IS A CANCER

Deconstruct the term Multiple Myeloma.

1. Normally plasma cells (developed from B cells and secrete immunoglobulins) selectively produce immunoglobulins on as-needed basis

2. With MM, some of plasma cells become malignant and overproduce immunoglobulins (too many antibodies!!!)

3. These immunoglobulins infiltrate mainly bones (and certain organs too), causing multiple malignant tumors that increase OSTEOCLASTIC activity

Explain the Pathogenesis of Multiple Myeloma.

1. Osteoclasts are hematopoietic cells that normally migrate along bone capillary walls and "chew up" bone cells when calcium's needed in body

2. In MM, osteoclasts go into OVERDRIVE and chew up more bone cells

3. Thus, bones get weaker (osteoporosis) and HYPERCALCEMIA occurs

Review what osteoclastic activity is and its relation to Multiple Myeloma.

1. Osteoporosis --> pathological fractures (bone breakage occurs w/ minimal stimulus)

2. Hypercalcemia with S&S of confusion, lethargy, weakness, kidney stones, & kidney failure

S&S of Multiple Myeloma?

Chemotherapy, radiation, bone marrow transplant

What's the treatment of Multiple Myeloma?

leads to HYPERpolarization, leading to HYPOactivity of muscles

REVIEW: Hypercalcemia leads to ___ which leads to ____

quantitative (numbers) , qualitative (quality/function of cell), OR BOTH

Problems with erythrocyte function can be what 3 things?

quantitative (too few)

Sickle Cell is an example of what kind of erythrocyte abnormality?

1. Less than normal number of total circulating erythrocytes AND/OR decrease in quality/quantity of Hgb

2. Anemia is the RESULT of a dz process—it's a state/condition and/or can be a SIGN of a dz but only in certain cases is it considered a dz process in itself

Again, describe what Anemia is.

1. Decrease in erythropoiesis like bone marrow dysfunction, leukemias, or hypersplenism

AND/OR

2. Loss of RBCs via hemorrhage and/or pathologic hemolysis

Anemia can be a result of disorders that precipitate...

1. Shape....abnormal shape duh (ex. sickle cell)

2. Substance...if reduced Hgb content, anemia may be called Hypochromic (less color)

Classifications of Anemias are according to changes in shape, substance, or size of RBC. Define shape and substance changes and give examples of each.

1. Microcytic (tiny cell)

2. Normocytic

3. Macrocytic (big cell)

What are the 3 categories of SIZE anemia?

1. Fewer and/or deranged RBCs or Hgb means disruption in effectiveness of O2 reaching cells of tissues & organs for use in cellular metabolism

2. Therefore, anemic patient will usually have S&S related to hypoxemia (low oxygen in blood)

3. These S&S can be slow & subtle if there's slow process and body compensates OR immediate and severe if cause is sudden

What are the S&S of most types of anemia in general?

1. SOB

2. Weakness & fatigue, muscle cramps

3. CNS effects: slowed mentation, dizziness, lethargy, syncope (fainting)

4. Pallor (pale)—less rbcs traveling in skin capillaries means less pinkness

Reduced blood O2 (hypoxemia) --> tissue hypoxia --> what 4 things?

CBC (complete blood count)

- Look at RAW NUMBER and SIZE of cell

Diagnosis of an anemia AND its classification by what blood test? What do we look at to classify an anemia?

1. Look at RBC count

2. To NAME type of anemia, look at MCV (mean corpuscular volume) aka size of each RBC

What parts of the CBC do we use to classify Anemias?

1. Low Hgb (normal is 14-18 gm/L)

2. Low hematocrit (Hct) aka % of RBCs in lood (42 to 52% is normal)

What other numbers might you see in an anemic person's CBC?

1. CBC...RBCs under 4million --> anemia

2. Look at size (MCV) and categorize as either micro, macro, or normocytic

3. Look at Hgb and Hematocrit

4. Know the dz processes and treatments that correlate to each category of size

Okay now just give a quick step by step on how to label an Anemia. (4 steps)

RBC = 4-6mil

MCV - 80 to 95

RBC and MCV norm?

Anemia where MCV smaller than normal

- CBC shows RBCS less than 4 mill and MCV less than 80

What's Microcytic Anemia?

1. Something causes body to slowly, chronically, lose RBCs thus begins to "run low" on material to make enough full sized RBCs

2. Over time, bone marrow tries to catch up by churning out RBCs that are smaller

3. This compensatory response isn't enough to bring RBC numbers up to normal but better than nothing

Explain the patho of Microcytic Anemia.

1. Women w/ heavy menses

2. Occult (hidden) GI bleeding

What are 2 examples of chronic, steady loss of RBCs?

- Iron deficiency anemia

- Because iron (Fe) is an essential part of HgB molecules which are lost with the RBCs

Sometimes Microcytic Anemia is called....? Why?

- In common with MOST anemias are: RBC

What are the S&S of Microcytic anemia?

stop blood loss if possible and give iron supplements

Treatment of Microcytic Anemia?

anemia where size of RBC is normal...RBC lower than 4 mil and MCV normal

What's Normocytic Anemia?

1. Something causing RAPID blood loss so no time for compensatory response of smaller RBCs (ex: traumatic hemorrhage)

2. Chronic disease cause slow down in RBC production—produced in normal size but slowly and in less numbers (ex: AIDS, lupus, chronic renal failure)

What are the 2 possible pathos of Normocytic Anemia?

- In common with MOST anemias are: RBC

S&S of Normocytic anemia?

1. For acute blood loss—stop blood loss if possible and give units of blood as needed to replace

2. For chronic disease—give type of growth hormone called Erythropoietin to stimulate bone marrow to make RBCs

Treatment of Normocytic Anemia?

anemia where MCV (size) larger than normal so CBC shows RBCs of less than 4 mil and MCV above 95

What's Macrocytic Anemia?

- Certain dz processes cause faulty DNA coding of RBC size so that RBCs are larger than normal

- Most common example of macrocytic anemia is PERNICIOUS ANEMIA

What's the patho of Macrocytic Anemia?

1. 1. Mostly seen in elderly, patients w/ GI absorption disorders, and people w/ inadequate nutrition (alcoholics)

2. Often begins w/ diminished production of INTRINSIC FACTOR...hormone made by parietal cells in stomach

3. W/o intrinsic factor, vitamin B12 can't be adequately absorbed from food

4. W/o vitamin B12, DNA malfunctions & makes coding error in RBC creation --> too few RBCs made AND they're larger than normal

Describe Pernicious Anemia (4 bulletpoints).

- In common with MOST anemias are: RBC

S&S of macrocytic anemia?

- Often described as pins and needles or burning

- Caused from lack of B12 as an important nerve mediator

Describe Neuropathies such as parasthesia.

usually injection of B12

Treatment of Macrocytic anemia?

disorder of more than normal number of total circulating RBCs (erythrocytosis)

What are Polycythemias?

primary & secondary

What are the 2 forms of Polycythemia?

1. AKA polycythemia vera—rare condition where there's slow development of hyper-proliferation of bone marrow stem cells

2. Removal of 300-500ml blood 3 to 4 times per month

1. What is Primary Polycythemia?

2. What is its treatment?

- Compensatory increase in RBCs in response to hypoxic conditions

- Mechanism of compensation is low O2 --> body increase erythropoietin secretion --> more RBCs made

What is Secondary Polycythemia?

1. Living at higher altitudes (> 10k feet)

2. Smoking (increased lvl of CO2)

3. Chronic low-O2 conditions like COPD

What are 3 examples of hypoxic conditions?

RBCs above 6 million, Hematocrit above 52%

Polycythemia is characterized by what numbers of RBCs and Hematocrit?

1. Problems related to overproliferation of RBCS most often due to "thickness" of blood...extra RBCs = sludge-like blood

2. Sequelae of above is high risk for distal tissue ischemia since blood that's thick and slow moving (stasis) can increase chance of clots & blocking blood vessels

3. Thickened blood can cause extra workload on heart & lungs...higher risk of organ failure

Describe the Patho of Polycythemia.

1. Ischemic pain

2. Formation of blood clots

3. Heart & lung failure related issues

Based on the patho of polycythemia, what are the 3 S&S of polycythemia?

they'll be similar, relating to "easy bleeding" which can be frank (obvious) or occult (hidden)

For ANY kind of bleeding deficiency, S&S will be...

obvious or easily visible bleeding

What is Frank Bleeding?

1. Petechiae—pinpoint read spots that don't blanche

2. Purpura—larger areas that look purplish

3. Ecchymosis—general term for collection of blood under skin greater than ~1cm

What are the 3 types of under the skin frank bleeding?

1. Stools can be bloody or black and tarry

2. They can bleed...example is nose bleed

1. Describe GI bleeding.

2. Describe Mucous Membrane bleeding.

In GI tract when bleeding is slow and not in areas where blood can be digested, stools may look normal but have blood IN them.

Give an example situation of Occult (hidden) bleeding.

Platelets & factors

What are the 2 subcategories of clotting deficiency?

Thrombocytopenia

1. Congenital problems (rare)

2. Nutritional

3. Certain drugs & chronic dz

4. Autoimmune

Platelet (thrombocyte) deficiency is called what?

- What are its 4 causes?

1. IgG attacks platelets, diminishing their effectiveness

2. Called Idiopathic Thrombocytopenia Purpura (ITP)

Explain the Autoimmune cause of Thrombocytopenia.

1. Usually platelet count on CBC less than 100k (normal is 150 to 400k with average at 250k)

2. Bleeding as noted previously

What is the S&S of Thrombocytopenia?

refers to several possible different hereditary deficiencies of coagulation factors, usually X-linked recessive problem

Hemophilia is a clotting factor deficiency dz. What is it?

spontaneous bleeding or bleeding that's out of proportion to amount of trauma

S&S of Hemophilia?

1. Transfuse plasma—it has clotting factors

2. Transfuse concentrates of whichever clotting factor is deficient

2 Treatments for Hemophilia?

an inherited disorder like hemophilia but instead of clotting factors being low, tissue substance is low

What is von Willebrand disease (vWD)?

1. vWF is released by injured tissue

2. It "calls" platelets and binds to them and to fibrinogen to promote clotting—a "platelet plug"

von Willebrand Factor (vWF) is found in blood and tissue in various forms. Give one example.

it's the most common clotting disease...every 1 in 100 person has a variation of it

How common is vWD?

1. Mostly very mild clotting deficiency with mild incidences of easy bleeding

2. Ex. some women manifest very heavy periods due to a minor of vWD

S&S of vWD? (various types of this dz cause varied severity)

usually none needed, or symptomatic; certain more severe forms receive transfusions of vWF

Treatment of vWD?

1. Liver is site of protein synthesis, including coagulation factors

2. Liver dz --> coag factors --> decreased ability to clot --> easy bleeding

How can Liver diseases (like cirrhosis) can be a clotting deficiency dz?

1. Platelet counter greater than 400k

2. Can be from genetic myeloproliferative disorder or from secondary cause like RA, cancer, or after splenectomy

3. Causes hypercoagulation which can manifest as thrombotic disorder—proliferation of blood clot formation

Thrombocytosis is an "overactive clotting" dz. Describe it.

any combination of thrombocytosis and clotting factor overactivity

What are Thromboembolic disorders?

1. Thrombus—an arterial or venous clot attached to vessel wall

2. Embolus—an arterial or venous thrombus that's broken loose and travels in circulatory system

1. What's Thrombus?

2. What's Embolus?

Enlargement of spleen

1. Hematologic

2. Infectious

3. Congestive

4. Malignancies

5. Physiologic

What's Splenomegaly?

- What are the 5 categories of splenomegaly causes?

anytime there's increased hemolysis of RBCs, spleen can enlarge bc it has more RBC debris to process

(ex: Rh blood type incompatibility like erythroblastosis fetalis or ABO blood type incompatibility)—baby would be anemic AND have splenomegaly in erythroblastosis fetalis

What is a Hematologic cause of Splenomegaly?

1. Drug reactions and autoimmune disease in which theres a triggering of antibody attack on RBCs

2. Infections where RBCs are attacked like MALARIA

Hemolytic problems come from what 2 things?

1. Viral—heptatisi, mononucleosis (Epstein-Barr virus), CMV

2. Bacteria—TB

Splenomegaly can be caused by infection/inflammation. Describe.

Especially with leukemia...large numbers of WBCs coming through spleen

Explain how Malignancies can cause Splenomegaly.

usually individualistic quirk...idiopathic etiology & no S&S

What is Physiologic Splenomegaly?

1. Palpably large spleen (normally unable to palpate it)

2. May have pain

3. May have hypersplenism

3 S&S of splenomegaly?

1. Condition resulting from splenomegaly...large spleen means RBCs, WBCs, and thrombocytes/platelets become trapped there (sequestered)

2. All cells begin to be "chewed up", destroyed en masse...

What's Hypersplenism?

1. Spleen becomes more engorged & enlarged

2. Pancytopenia results...low numbers of ALL cells in blood

What are the 2 sequeale of all cells beginning to be chewed up and destroyed en masse?

anemia, increased risk of infection, "easy bleeding"

S&S of Pancytopenia?