Ipapburke Hematology Test 1- Normocytic

Front 1

MCV in normocytic anemia

Back 1

80-100

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Possible underlying mechanisms for normocytic anemia (3)

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inflammation, hemolysis, acute blood loss

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If the rate of change of the hematocrit/hemoglobin is slow in normocytic anemia, think what?

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Anemia of Chronic Disease (ACD)

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If the rate of change of the hematocrit/hemoglobin is fast in normocytic anemia, think what?

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hemolysis

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Possible signs of hemolysis (5)

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jaundice, icterus, HSM, increased reticulocytes, decreased haptoglobin

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If there is evidence of hemolysis in normocytic anemia, what should you look for on blood smear?

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shistocytes or spherocytes

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Differential Diagnosis for Normocytic anemia (5)

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ACD, acute blood loss, multifactorial anemia, renal failure associated anemia, aplastic anemia, or hemolysis

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Two types of hemolytic anemia

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intrinsic and extrinsic

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Another name for anemia of chronic disease

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anemia of inflammation

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2nd most common type of anemia

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Anemia of Chronic Disease (ACD)

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Anemia of Chronic Disease usually presents as normocytic, but may also present as?

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microcytic

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What is the key pathology behind anemia of chronic disease?

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disturbance in iron homeostatis as a result of chronic inflammation which leads to increased uptake and retention of iron within macrophages as ferritin

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The net result of the two combined inflammatory processes involved in ACD is what?

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decreased iron availability in the bone marrow which leads to less RBC being produced as well as reduced RBC survival

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What is the broad definition of Multiple Medical Problems (MMP)?

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presence of several systemic conditions (including non inflammatory conditions) that mat also be a clinical attributable cause of ACD

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Symptoms in ACD

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typical anemia symptoms that are usually mild

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Treatment of ACD

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treat the underlying inflammatory process; iron replacement only if severe, but generally not recommended if there isn't an iron deficiency; blood transfusion PRN; EPO consideration

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In what patients with ACD is EPO considered in (as treatment)?

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cancer pts. w/ chemo tx, chronic kidney dz, HIV and myelosuppressive S.E. of therapy

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ACD often coexists with what other type of anemia?

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Iron def. Anemia

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Typical presentation of acute blood loss

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usually source of bleeding is evident, but may be masked in hospitalized pt. w/ unusual source

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Why is the CBC only somewhat accurate in a patient with acute blood loss who is being actively resuscitated?

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Because IV fluids and blood products are being added to restore lost blood volume, changing the values on the CBC (SN: If the CBC is done before resuscitation begins, it will show a more accurate picture of pre injury)

Front 21

Explain multifactorial anemia.

Back 21

Anemia with more than one cause, part, chronic normocytic anemias that are not easily explained.

Front 22

Why is normocytic anemia present in most patients with prolonged renal failure?

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Slow reduction in erythropoietin production with the end result being less production of RBCs in bone marrow

Front 23

What is the treatment for patients with renal failure associated anemia?

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routine use of synthetic erythropoietin agents (ESAs) to reach a goal of Hg/Hct of 11 and 33%

Front 24

Why is routine measurement of serum erythropoietin not extremely diagnostic for renal associated anemia?

Back 24

Because the EPO level will not fall until the Hg is below 10 and even below 10, the EPO level will still fluctuate

Front 25

Aplastic anemia, Myelodysplasia, Acute leukemia, Myelofibrosis, magaloblastic anemia, and infiltrative dzs (lymphoma, myeloma) are examples of what broad group of D/O that cause pancytopenia?

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bone marrow disorders

Front 26

Give three examples of non-marrow disorders that can cause pancytopenia.

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hypersplenism, SLE, infection (TB, AIDS, leishmaniasis, brucellosis)

Front 27

What is the mechanism of primary aplastic anemia

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probably immune related, but not proven

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Types of secondary aplastic anemia (2)

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inherited (Fanconi's) and Acquired (chromo/radiation, drugs, infections, PNH (paroxysmal nocturnal hemoglobinuria)

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What is PNH?

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paroxysmal nocturnal hemoglobinuria

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What is Fanconi's Anemia?

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Autosomal recessive trait that mainly affects the bone marrow. It results in decreased production of all types of blood cells.

Front 31

MC characteristic physical anomalies in Fanconi's Anemia

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skin pigment changes, short stature, upper limb abnormalities, hypogonadal and genitalia changes (male mostly)

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Pancytopenia- definition

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medical condition in which there is a reduction in the number of red and white blood cells, as well as platelets.

Front 33

Explain bimodal distribution of aplastic anemia.

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occurs between ages 15-25 and again after 60 usually

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Presenting symptoms of aplastic anemia.

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bleeding, anemia, infections, routine CBC

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How is the diagnosis of aplastic anemia made?

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CBC and hypocellular bone marrow

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Treatment of aplastic anemia in people under 20

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bone marrow transplant

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Treatment of aplastic anemia in patient over 45

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usually immunosuppression

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Why is immunosuppression usually used to treat aplastic anemia in patient over 45?

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due to increased risks with graft-versus-host disease

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Prognosis for patient under 50 with aplastic anemia

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70% 5 yr survival

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Prognosis for patient over 70 with aplastic anemia

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50% 5 yr survival

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What is the one year untreated mortality rate for patients with severe aplastic anemia?

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70% mortality rate within 1 year

Front 42

What is the etiology of PNH?

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acquired hemolytic anemia caused by a hematopoietic stem cell precursor mutation defect in PIGA gene that cause RBCs to lyse via complement system

Front 43

Is PNH considered intrinsic or extrinsic hemolytic anemia?

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intrinsic

Front 44

Patients with PNH are deficient in what two factors?

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CD55 and C59 which regulate complement response

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When does PNH progress from episodic hemolysis to aplastic anemia?

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When stem cell injury occurs beyond just the PIGA mutation

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Clinical Signs of PNH

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cola colored urine, hemosiderin always present in urine sediment, hemosiderin deposits in kidneys on CT/MRI

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Hallmarks of intravascular hemolysis

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elevated retic count and elevated serum LDH w/ low serum haptoglobin (w/o HSM)

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Veins where thrombosis may occur in PNH (considered "unusual locations")

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hepatic, abdominal, cerebral, or sub dermal veins

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Tx of PNH

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Eculizumab (anti complement agent) lifelong; adjunct therapy- anticoagulation, iron supplement PRN

Front 50

What is intrinsic hemolytic anemia?

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due to defect in RBC itself

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What is extrinsic hemolytic anemia?

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hemolysis is caused by an external factor itself

Front 52

3 categories of intrinsic hemolytic anemias.

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Membrane defects, oxidative vulnerability, hemoglobinopathies

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4 categories/causes of extrinsic hemolytic anemias.

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immune, microangiopathic, infection, burns/ hypersplenism

Front 54

Is haptoglobin increased or decreased in hemolytic anemias?

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reduced- because it binds with the free hemoglobin that is released in the serum during hemolysis

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Is indirect bili increased or decreased in hemolytic anemia?

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increased with significant intravascular hemolysis

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Is LDH increased or decreased in hemolytic anemia?

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increased- it is released from lysed RBCs

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Is the reticulocyte count and RPI increased or decreased in hemolytic anemia?

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usually increased (normal or low if coexisting hypoproliferative d/o of bone marrow)

Front 58

What is the cause of hereditary spherocytosis?

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It is a chronic hemolytic anemia caused by RBC membrane defect in spectrin

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How do you distinguish between Autoimmune Hemolytic Anemia and Hereditary Spherocytosis?

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negative Coombs' test

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The reason why hereditary spherocytosis may go undetected until adulthood?

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due to variable penetrance

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What test is performed sometimes to diagnose Hereditary Spherocytosis when Coombs is negative and hemolysis is present?

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Osmotic fragility test

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What does an abnormal Osmotic fragility test indicate?

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an increased presence of spherocytes (test may be falsely negative in mild HS)

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Is G6PD deficiency an intrinsic or extrinsic defect?

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intrinsic

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What is oxidative stress?

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cellular damage that occurs from free radicals that cannot be fully neutralized by antioxidants

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What is the most frequent cause of oxidative stress?

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medications and infections

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What is G6PD Deficiency?

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hereditary (X-linked) enzyme deficiency that results in hemolysis with oxidative stress

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What is the importance of G6PD's function in keeping gluthathione in a reduced state?

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protects hemoglobin from oxidative denaturation

Front 68

What population has a higher % of reduced G6PD activity?

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African Americans

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Why can lab measures of G6PD be falsely normal is measured right after a hemolytic episode?

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Because G6PD activity declines in older RBCs so older blood cell populations are affected. After hemolysis, it is younger RBC with more G6PD activity left

Front 70

What are Heinz Bodies?

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oxidized hemoglobin that has formed precipitants that can be seen on peripheral smear

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What classes of drugs may induce oxidative stress?

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antimalarials (primaquine, dapsone), sulfonamides, analgesics

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Best tx for G6PD Def.

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avoid oxidative stress

Front 73

Autosomal recessive d/o caused by a point mutation in the B globin chain gene that causes Hem S to form

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Sickle Cell Anemia

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Populations most affected by sickle cell

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Africans (some others in Malaria belt); African Americans

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What are Sickle "Crises"?

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Sickle Cell anemia has an episodic course- episodes are "crises"

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Lab Findings with Sickle Cell

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chronic normocytic anemia; chronic reticulocytosis (even higher with crises)

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What is Acute Chest Syndrome?

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found in Sickle Cell- fevers, chest pain, hypoxemia, and pulm. Infiltrates

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Tx for Sickle Cell anemia

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supportive care (O2, fluids, analgesia), hydroxyurea (to incr. HgB F), transfusions

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What is Autoimmune Hemolytic Anemia caused by?

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extrinsic defect caused by acquired antibodies to a component of the RBC membrane

Front 80

What determines the severity of AIHA?

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amount/type of Ab, activity of Ab, Ab ability to fix complement

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Most common AIHA

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warm

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Antibodies involved in warm AIHA

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IgG Ab that react to normal body temps

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Usual result in warm AIHA

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results in extravascular hemolysis (spleen)

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Antibodies involved in Cold AIHA

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IgM that only reacts at reduced body temperature

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Usual result in cold AIHA

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intravascular hemolysis (liver)

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Is complement activity increased or decreased in "cold" AIHA?

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increased

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Etiology of warm AIHA

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idiopathic (majority); lymphomas, SLE/autoimmune d/o's; drugs, post viral infections

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Etiology of cold AIHA

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lymphoproliferative dz, infections (mycoplasma, Mono-EBV)

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Most pertinent clinical feature of AIHA

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episodic course usually ass. w/ physiologic stress

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What is seen on smear of AIHA?

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significant spherocytes

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Why is warm AIHA usually more severe?

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more frequent activity

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What does the severity of cold AIHA depend on?

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the temperature

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Tx of warm AIHA

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immunosuppression, complicated transfusions, splenectomy

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Tx of cold AIHA

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avoidance

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Intravascular hemolysis due to RBC fragmentation (trauma) that results in schistocytosis and thrombocytopenia

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Microangiopathic Hemolytic Anemia (MAHA)

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What is often the role of infectious agents in hemolytic anemia?

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the infectious agent can act as a facilitator to other hemolytic processes

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How does aplastic anemia present?

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As pancytopenia

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How are hemolytic anemias recognized?

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by reliable, rapid hematocrit drop in the absence of bleeding

Front 99

List intrinsic hemolytic defects

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HS, G6PD, sickle cell

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List extrinsic hemolytic defects

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AIHA warm and cold