Anemia

Question 1

Red blood cells are produced through_______? Production is stimulated by the hormone _____, synthesized in the _______. Immature erythrocytes are call______?

Answer 1

Erythropoiesis, erythropoietin (EPO), kindey, reticulocytes.

Question 2

Value for hemoglobin, hematocrit, RBC, MCV, reticulocyte count?

Answer 2

Hgb: M-13.5-17.5 F-12-16. Hct: M-45, F-35, RBC: M-4.5-6 F-4-5.4 MCV- 81-90, recticulocyte count 20,000-100,00

Question 3

What is anemia?

Answer 3

Significant reduction in mass of circulating RBCs which causes O2 binding capacity of blood to diminish. Anemic pts can have decreased RBCs or hemoglobin

Question 4

What is microcytosis? Causes of microcytosis?

Answer 4

Small erythrocytes caused by abnormal erythroid maturation

Question 5

What is and causes macrocytosis?

Answer 5

Increase in size of erythrocytes caused by abnormal erythroid maturation, MCV >100. RBC aplasia, alcoholism, aplastic anemia, myelodysplastic syndromes, hemolytic anemias

Question 6

Causes of normocytosis?

Answer 6

ACD, acute hemorrhage, hemoglobinopathies, primary bone marrow (decreased erythroid progenitors), secondary to chronic disease

Question 7

Symptoms of anemia?

Answer 7

Often asymptomatic. Fatigue, dyspnea, pallor, palpitations, decreased exercise tolerance.

Question 8

Features of decreased red blood cell production?

Answer 8

Onset is insidious, reticulocyte count is inappropriately low, bone marrow examination is often required for diagnosis, MCV are informative

Question 9

Features of increased red blood cell destruction?

Answer 9

Onset is abrupt or insidious, reticulocyte count is increased, red cell morphology on peripheral smear is usually informative, dont need BM biopsy

Question 10

Test for anemic workup?

Answer 10

CBC, peripheral blood smear, bone marrow examination

Question 11

Significant increase in reticulocyte on CBC count could indicate?

Answer 11

Hemolytic anemia

Question 12

A low reticulocyte count on CBC suggest what mechanism for anemia?

Answer 12

Failure to produce RBCs

Question 13

What are some signs of acute anemia due to blood loss?

Answer 13

Increase of pulse of 25% or more or a fall in systolic BP of 20mm Hg when going from supine to sitting (indicating hypovolemia), MCV will increase initially due to young RBC, increase leukocytosis and “left shift” in WBC due to stress, retics increase after a few days.

Question 14

Causes of anemia due to chronic blood loss?

Answer 14

Usually d/t lesions in GI tract or uterus. Need to test stool specimens fo occult blood

Question 15

How do endocrine disease cause anemia and what are some of the diseases?

Answer 15

Hormones help stimulate erythroid cells causing mild-moderate anemia. Hypothyroidism, hypoadrenalism, hypogonadism, hypopituitarism

Question 16

Disease that can cause anemia of chronic disease?

Answer 16

Renal insufficiency, aging, endocrine disease, liver disease, inflammatory disease

Question 17

Risk factors that can cause anemia of chronic disease (ACD)?

Answer 17

Chronic inflammatory conditions like autoimmune d/o (SLE, RA, sarcoidosis, vasculitis, IBD), Cancer, CKD, infection, chronic rejection after organ transplant

Question 18

Work up for ACD?

Answer 18

CBC, reticulocyte count and index, peripheral blood smear, serum iron lvl, TIBC, transferrin saturation, ferritin, EPO lvl

Question 19

What would the test results look like for ACD?

Answer 19

Hgb-8.5-9, low retic ct (ineffective erythropoiesis), low serum iron and transferrin, serum ferritin normal or increased, smear will have hypochromic normocytic RBCs

Question 20

Treatment for ACD?

Answer 20

Treat underlying disease. Acute-transfusion saved for severe anemia. Chronic- Erythropoiesis-stimulating agents (ESA) (for CKD, chemotherapy, zidovudine), possible supplemental iron

Question 21

Possible complications of ESA?

Answer 21

High dose ESA in CKD- increase CV and thromboembolic events. ESA in cancer correlated with tumor progression and mortality.

Question 22

What is aplastic anemia?

Answer 22

Bone marrow failure syndrome defined by peripheral blood pancytopenia and hypocellular bone marrow aplasia

Question 23

What are some of the clinical presentations of aplastic anemia?

Answer 23

Mucosal bleeding, easy bruising, petechiae, heavy menstrual bleeding secondary to thrombocytopenia. Fatigue, lasstitude, pallor, DOE, palpitations secondary to anemia. Fever and sore throat due to neutropenia. Can have short stature, skeletal/nail changes.

Question 24

What test would you order for AA workup and what would you see on test?

Answer 24

BM aspiration or biopsy (dec. erythropoietic and myelopoietic precursor cells), CBC ( pancytopenia, macrocytic, toxic granulation of neutrophils, reticulocytopenia) HAM test, MRI ( high fat content in bone marrow)

Question 25

Treatment for acute moderate AA?

Answer 25

Anti-thymoctye globulin ATG (kills T lymphocytes allowing bone marrow to resupply stem cells). Non-responders- Cyclosporine A (prevent T-lymphocyte activation), androgen

Question 26

Treatment of acute severe AA?

Answer 26

Kids/young adults- Human leukocyte antigen (HLA) typing for BMT- if none treat with CSA and ATG. Older adults- ATG and CSA. No response- non match BMT, androgens, 2nd immunosuppresent

Question 27

Treatment for chronic AA?

Answer 27

Allogenic BM transplantation. ATG. ISAs (cyclosporin, cyclophosphamide, corticosteroids). Androgen (danazol). Oral thrombopoietin mimetic (eltrombopag)

Question 28

What is hemolytic anemias?

Answer 28

Anemia resulting from an increased rate of erythrocyte destruction in peripheral circulation.

Question 29

What is an abnormality of the RBC membrane, which make the RBC unusually sensitive to lysis by complement?

Answer 29

Paroxysmal nocturnal hemoglobinuria. Acquired disease

Question 30

What is a congenital defect of erythocyte cell membrane, becoming abnormally permeable to Na, resulting in thickened and almost spheric erythrocytes that are fragile and susceptible to spontaneous hemolysis?

Answer 30

Hereditary spherocytosis.

Question 31

Disease where RBC appear spiculated and are destroyed prematurely, predominantly in the spleen?

Answer 31

Spur cell anemia

Question 32

HIV can induce which type of AIHA?

Answer 32

Warm and cold AIHA "mixed-type"

Question 33

What type of antibodies are involved in warm antibody mediated AIHA? Where does destruction take place?

Answer 33

IgG which are characterized by phagocytosis of RBC. It allows phagocytic cells to bind to complex. RBCs are destroyed in the spleen. Associated with AI diseases.

Question 34

What type of antibodies are involved in cold antibody mediated AIHA? Where does destruction take place?

Answer 34

IgM is potent activator of complement pathway which caused complement mediated lysis of RBCs. IgM AIHA takes place in Kupffer cells of liver.

Question 35

Phagocytic AIHA is termed ________, while complement mediated lysis of RBCs is termed _______ AIHA which requires overwhelming ______ activation?

Answer 35

extravascular, intravascular, complement activation.

Question 36

Test performed to determine which autoantibody is causing AIHA?

Answer 36

Coombs test, aka antiglobulin test. There are indirect and direct types of Coombs test.

Question 37

Clinical presentation of AIHA?

Answer 37

Fatigue, dyspnea, pallor, jaundice, tachycardia with flow murmur (pronounced anemia). Intravascular hemolysis may present with dark urine and back pain. Hepatomegaly, lymphadenopathy, splenomegaly may be present

Question 38

Lab test for AIHA and expected results?

Answer 38

CBC (reticulocytosis, low haptoglobin) CMP (elevated indirect bilirubin, elevated LDH), peripheral smear, Coombs (direct antiglobulin test), Hep serology, ANA, UA for hemosiderinuria and hemoglobinuria, CXR (rule out lymphoma), CT chest, abdomen, pelvis (r/o lyphoma)

Question 39

DAT results: +IgG alone or with IgG+ C3D, which type of AIHA?

Answer 39

Most likely due to warm AIHA

Question 40

DAT result: + C3d alone

Answer 40

Most likely due to cold AIHA

Question 41

Treatment for acute and chronic cold AIHA?

Answer 41

Acute: transfusions of prewarmed PRBCs, antibxs, +/- corticosteroids, supportive care. Chronic: supportive and symptomatic, avoid cold, treat febrile infections, +/- vaccines

Question 42

Treatment for warm AIHA?

Answer 42

Prednisone 1-2mg/kg/day w/ Danazol. Splenectomy if inadequate response to steroids. ISA if the above fail (Azathioprine, cyclophosphamide, cyclosporine, rituximab)

Question 43

At what value would you see cardiac symptoms such as increased CO, HR, SV

Answer 43

Hemoglobin less than 7.5