1
Q
Define polycythemia.
A
Increase in the total number of RBCs, amount of Hgb, and RBC mass in circulation.
2
Q
Define poikilocytosis.
A
Increase in reticulocytes in the peripheral bloodstream due to premature release.
3
Q
What are the 4 components of whole blood?
A
- Plasma
- RBCs
- WBCs
- Platelets
4
Q
What primarily composes plasma?
A
- Water
- Salt
5
Q
Where is the primary origin of RBCs? Extramedullary sites?
A
Primary: Bone marrow
Extramedullary: liver and spleen
6
Q
What is the primary stimulatory hormone for RBC production and where is it primarily made?
A
EPO/erythropoietin, made in the kidneys.
7
Q
What is the average lifespan of a RBC?
A
120 days/4 months.
8
Q
What is the primary stimulatory hormone for platelet production and where is it primarily released from?
A
Thrombopoietin, released by the liver and kidney.
9
Q
What is the average lifespan of a platelet?
A
7-10 days.
10
Q
What is responsible for the difference in normal RBC ranges between sexes?
A
Production of testosterone. Males have a higher average range.
11
Q
What natural factors might increase hemoglobin concentration? Decrease?
A
Increase: Smoking, high altitudes, males, young infants.
Decrease: Female and pregnancy.
12
Q
How can Hct be calculated from Hgb?
A
Hct = Hgb x 3
13
Q
Define anisocytosis.
A
Increased RDW with reticulocytosis.
14
Q
What does increased MPV suggest?
A
New platelets are being formed, since new platelets are larger.
15
Q
What minerals/vitamins are essential to normal RBC production? Why?
A
B12/Folate: Proliferation and maturation.
Iron: Accumulation of Hgb.
16
Q
What findings concerning a RBC would suggest it is a reticulocyte?
A
- Presence of RNA
- Bluish or lack of biconcavity
- Larger size
17
Q
What general conditions can decrease erythropoiesis?
A
- Nutrient deficiencies
- Bone marrow disorders/suppression
- Low levels of trophic hormones
- Acute/chronic inflammation
18
Q
What two conditions can result in increased RBC destruction/clearance?
A
- Hemolytic anemias
- Hypersplenism
19
Q
What is the most common cause of anemia?
A
Blood loss.
20
Q
What common social history factor can induce anemia? What anemia does it primarily result in?
A
Alcohol abuse, which leads to folate deficiency anemia.
21
Q
What conditions most commonly result in microcytic anemias? What is the primary pathophysiology behind microcytic anemias?
A
Sickle Cell Disease
Alpha/Beta Thalassemia minor
Sideroblastic anemia
Most commonly due to decreased Hgb content within the RBC.
22
Q
If a normocytic anemia is present, what is the most probable next step following a CBC?
A
Peripheral smear to observe the actual morphology.
23
Q
What conditions most commonly result in normocytic anemias?
A
- CKD
- Anemia of chronic disease/inflammation
- Mild iatrogenic anemias
- Acute blood loss
24
Q
What conditions most commonly result in macrocytic anemias?
A
- Alcohol abuse
- Folate/B12 deficiency
- Myelodysplastic syndromes
- AML
- Reticulocytosis induction
- Drug-induced anemia
- Liver disease
25
What is the underlying cause for the symptoms of anemia?
Impaired oxygen delivery to tissues.
26
How does the body compensate for anemia?
* Increasing O2 extraction
* Increasing SV and HR
27
What clinical findings strongly point to anemia?
* Fatigue
* Tachycardia at rest
* Bounding pulses
* Pallor
28
What physical exam findings might suggest anemia?
* Pallor
* Jaundice
* Petechiae
* Bruising
* Pale conjunctiva
* Scleral icterus
* HSM
* Bony tenderness along the sternum or anterior tibia
29
If a patient presents with a microcytic anemia, what is the next step in evaluating them?
Iron studies. (Fe, TIBC, ferritin)
30
If a patient presents with a microcytic anemia with no dysplasia or drug use, what is the next step in evaluating them?
* Serum B12/Folate levels.
* MMA/Homocysteine (Potentially)
31
A patient presents with a macrocytic anemia and is a known vegetarian that takes no supplements. What secondary labs should we order and what findings would we probably expect?
* Low serum B12
* Elevated MMA and homocysteine.
32
A patient presents with a macrocytic anemia and is known to not eat their veggies. What secondary lab should we order and what findings would we probably expect?
* Low folate
* Elevated homocysteine
* Normal MMA.
33
A patient presents with a microcytic anemia and additional iron studies were ordered. They have a low serum Fe, high TIBC, and a low ferritin. What is the most likely cause for their anemia?
Iron deficiency anemia.
34
A patient presents with a microcytic anemia and additional iron studies were ordered. They have a low serum Fe, normal TIBC, and a normal ferritin. What is the most likely cause for their anemia?
Anemia of chronic disease/inflammation.
35
A patient presents with a microcytic anemia and additional iron studies were ordered. They have an elevated serum Fe, normal TIBC, and elevated ferritin. What additional lab should we consider ordering?
Peripheral smear. (Checking for sideroblasts, teardrop cells, or target cells)
36
A patient presents with a microcytic anemia and additional iron studies were ordered, along with a peripheral smear. The findings are as follows: elevated serum Fe, normal TIBC, elevated ferritin, teardrop red cells, and target cells. The patient’s family history is also positive for an inherited RBC disorder. What is the most likely cause for the anemia? What lab study could we order to confirm it?
Alpha/beta thalassemia, which would require a hemoglobin electrophoresis test.
37
A patient presents with a macrocytic anemia. What additional lab study should be ordered to help determine the underlying etiology?
Peripheral smear.
38
What condition MUST be considered when it comes to CBC interpretation?
Patient volume status, which can affect Hgb, Hct, and RBC counts. (All concentration measurements)
39
In an anemic patient, what is the preferred method for evaluating reticulocyte count?
Reticulocyte index (RI), which will adjust for altered hematocrit.
40
A patient presents with a normocytic anemia and increased relic count. What is the most likely underlying etiology and what labs should be ordered to further evaluate them?
1. Indicative of hemolysis.
2. Order Serum LDH, Indirect bilirubin, and serum haptoglobin.
3. Consider direct antiglobulin test.
41
What blood test is the most reliable indicator of total-body iron status?
Ferritin
42
A patient presents with macrocytic anemia. What lab tests should be order to further evaluate them?
* Retic Count
* B12
* Folate
43
What are the 3 underlying etiologies that result in megaloblastic anemias?
* Folate deficiency
* B12 deficiency
* DNA synthesis inhibitor use
44
What hallmark finding on peripheral smear suggests intravascular hemolytic anemia?
Schistocyte formation.
45
What hallmark finding on peripheral smear suggests extravascular hemolytic anemia?
Spherocyte formation.
46
What clinical finding can often differentiate extravascular hemolytic anemia from intravascular?
Hematuria is mainly only present in intravascular hemolytic anemia.
47
What lab finding should always be decreased in intravascular hemolytic anemia?
Serum haptoglobin.
48
What can result in a false elevation of serum haptoglobin?
Chronic inflammation.
49
What are the hallmark findings that suggest hereditary spherocytosis? (S/s & lab)
* Jaundice with splenomegaly, often post stressful event.
* Spherocytes (lack of central pallor)
* Potentially elevated MCHC
* Negative Coomb’s
50
What are the treatment options for hereditary spherocytosis?
* Folic acid 1mg daily.
* Definitive: Splenectomy
51
A patient is suspected to have a hemoglobinopathy. Hemoglobin electrophoresis is performed, with equal proportions of HbA, HbA2, and HbF noted. What is the most likely underlying etiology?
Alpha thalassemia.
52
How many alpha-globulin gene deletions are required for a patient to generally present with symptoms and abnormal CBC values?
2 deletions at minimum.
53
What findings would immediately suggest HbH disease in an alpha thalassemia patient?
Presence of a HbH band on electrophoresis.
Inclusion body presence on peripheral smear.
54
What peripheral smear findings are expected in a patient with alpha thalassemia trait?
* Hypochromic, microcytic cells.
* Target cells.
55
A mother presents with a fetus who was tested for alpha-globulin gene deletion due to family history. 4 gene deletions are present. What is the suggested treatment for this?
Pregnancy termination due to high maternal morbidity risk and low survivability of the fetus.
| Hydrops fetalis
56
What is a general monitoring tip for any patient being treated for suspected hemolytic anemia in regards to tranfusions?
If being given blood transfusions, iron overload is likely. Iron supplementation is generally only indicated if it is the hemolysis is intravascular.
57
A patient presents to the ER with anemia on CBC. A hemoglobin electrophoresis is ordered to help determine the underlying etiology. Increased proportions of HbA2 and HbF are found. What is the suspected underlying etiology?
Beta thalassemia.
58
What physical exam findings might suggest beta thalassemia specifically?
Chipmunk facies: thinning of long bones, pathologic fracture, flattened nasal bridge, and elongated cheeks, and failure to thrive.
Suggests moderate-severe beta thalassemia.
59
How are beta-thalassemia and alpha-thalassemia different on peripheral smear?
Generally similar. Beta-thalassemia major may present with additional nucleated RBCs.
60
A patient presents with beta-thalassemia major. Of note in their H&P is that they are a Jehovah’s witness. What are the suggested treatment options for the patient?
* Splenectomy
* Luspatercept
* Allogeneic bone marrow transplant (only in younger patients if splenectomy not chosen)
61
What is the primary underlying etiology for the symptoms of sickle cell disease?
Vaso-occlusive episodes due to sickle cell shaped RBCs getting stuck.
62
What is a hallmark lab finding that indicates sickle cell disease?
Presence of HbS band, as well as abnormal proportions of HbA, HbA2, and HbF.
63
What hemoglobin concentration is elevated in sickle cell disease? Why?
HbF is elevated due to the impaired function of HbA and HbA2.
| Body compensating for inefficient Hgb.
64
What elevated CBC value would easily differentiate sickle cell disease from thalassemias?
Elevated WBCs, usually 12k-14k in sickle cell disease.
65
Presence of what inclusion body in a peripheral smear is highly suggestive of sickle cell disease?
Howell-Jolly inclusion body, presenting as a very dark and purple spot.
66
What specific clinical findings on PE are highly suggestive of sickle cell disease?
* Dactylitis
* Retinal issues
* Cardiomegaly
* Hyperdynamic precordium
67
# ```
```
What is the most common clinical presentation of sickle cell disease and its main trigger?
* Sickle cell crisis induced by a stressor.
* Results in ischemia and resulting sudden pain that can resolve spontaneously.
68
What drug would be considered first-line treatment for a patient presenting with multiple sickle cell crises? What would contraindicate it?
Hydroxyurea to increase HbF levels.
Contraindicated if possibly pregnant due to teratogenicity.
69
A patient with persistent sickle cell crises is on hydroxyurea but does not like the side effects. What other second-line medications can be considered? What is the main prohibitory factor?
* Crizanlizumab/Adakveo to decrease episodes.
* L-glutamine (pharmaceutical grade).
| Both are extremely expensive.
70
An African-American patient presents with episodic pain, often lasting weeks, but suddenly resolves spontaneously. On physical exam, they are noted to have hepatomegaly and a hyper dynamic precordium. CBC reveals a microcytic anemia and leukocytosis. They are currently in an episode. They need immediate treatment. What is the treatment protocol?
* Hydration
* Oxygenation
* Pain control
* Splenectomy (if splenic sequestration crisis is present, indicated by Hgb drop of >2 from baseline and rapid splenomegaly.)
71
An African-American patient presents with episodic pain, often lasting weeks, but suddenly resolves spontaneously. On physical exam, they are noted to have hepatomegaly and a hyper dynamic precordium. CBC reveals a microcytic anemia and leukocytosis. They are currently in an episode. They have been treated, but express concerns that they want to be cured of this condition. What is the definitive treatment for their condition? What should be considered for them to receive it?
Allogeneic hematopoietic stem cell transplantation.
Must evaluate to see if end-organ damage has occurred, as the curative rate is much lower if end-organ damage is present.
72
An African American male presents to the clinic with episodic pain. No splenomegaly is present, and he notes that it tends to occur after he eats his favorite food, fava beans. He sometimes has hematuria as well. What lab should we order to diagnose him and what is the expected result?
G6PD assay, which should be elevated post episode.
73
What is the underlying pathology behind G6PD deficiency?
Oxidative stress denatures Hgb, resulting in Heinz bodies and splenic destruction or spontaneous destruction of RBCs.
74
Peripheral smear demonstrates bite and blister cells, along with reticulocytes. Upon special staining, Heinz bodies are noted as well. What condition might this patient have?
G6PD deficiency.
75
An African American male presents to the clinic with episodic pain. No splenomegaly is present, and he notes that it tends to occur after he eats his favorite food, fava beans. He sometimes has hematuria as well. What patient education should be provided to him?
* Avoid oxidative foods (Fava beans, soy, red wine)
* Avoid oxidative drugs (quinolones, aspirin, sulfas, Macrobid)
* Genetic counseling
* Folic acid supplementation
76
A patient presents with suspected autoimmune hemolytic anemia. What historical factor must be asked to determine the underlying etiology?
Drug history, as drug-induced immune hemolytic anemia is different from autoimmune.
77
What organ and its respective cells can be involved in RBC destruction besides the spleen in autoimmune hemolytic anemia?
Kupffer cells in the liver.
78
What peripheral smear findings suggest autoimmune hemolytic anemia?
Marked microspherocytosis.
79
What is first-line treatment for a patient with autoimmune hemolytic anemia?
Prednisone
| Steroids are generally indicated for autoimmune conditions
80
What is the classic case of hemolytic disease of the newborn or erythroblastosis fetalis?
Rh- mother with Rh+ father/fetus on 2nd child.
81
How do we test for erythroblastosis fetalis and the expected results?
* Newborn: Positive DIRECT Coombs test.
* Mother: Positive INDIRECT Coombs test.
82
A 29-year old female 10 weeks pregnant G2P1 presents to the clinic. The patient’s bloodwork reveals that she is Rh-. Upon further questioning, the father is noted to be Rh+. What tests should be ordered to confirm the suspected diagnosis and what is the treatment?
* Order direct Coombs for fetus and indirect Coombs for mother.
* Intrauterine fetal transfusion
* Early labor induction once fetus is viable
* Maternal plasma exchange
83
A 29-year old female G1P1 presents to the clinic. The patient is attempting to have a second child with her husband. She says that her bloodwork after her first pregnancy revealed her to be Rh-. Her fetus was Rh+. What would make us concerned for a second child’s viability and what is the suggested treatment?
Erythroblastosis fetalis is likely to occur with her 2nd child. She should be given RhoGAM if she wants to have a second child.
84
What is the underlying defect in paroxysmal nocturnal hemoglobinuria?
CD55 and CD 59 defects, resulting in hemolysis due to MAC formation.
85
A 25-year old patient suddenly presents with episodic morning hematuria. UA reveals that the presence of hemoglobin as well in their urine. They are admitted for overnight observation. Midnight labs reveal a pH of 7.2. What further diagnostics should be ordered for the patient to diagnose them?
Flow cytometry = gold standard
Urine hemosiderin = flow cytometry not available.
86
What is the classic triad that indicates paroxysmal nocturnal hemoglobinuria?
* Chronic intravascular hemolysis with recurrent crises
* Thromboses in unusual sites
* Cytopenias related to bone marrow failure
87
A 25-year old patient suddenly presents with episodic morning hematuria. UA reveals that the presence of hemoglobin as well in their urine. They are admitted for overnight observation. Midnight labs reveal a pH of 7.2. Further testing reveals positive flow cytometry. The patient’s condition worsens and 1 unit of PRBCs is transfused. What medications should be considered for their condition?
* Eculizumab, which is a C5 MAB.
* Corticosteroids to reduce hemolysis.
| PNH
88
A 25-year old patient suddenly presents with episodic morning hematuria. UA reveals that the presence of hemoglobin as well in their urine. They are admitted for overnight observation. Midnight labs reveal a pH of 7.2. Further testing reveals positive flow cytometry. The patient’s condition worsens and 5 unit of PRBCs are transfused. Their anemia is controlled but Hgb is still low. What is the definitive treatment option for their condition?
Allogeneic hematopoietic stem cell transplant.
## Footnote
Paroxysmal nocturnal hemoglobinuria.
Also the same definitive treatment for SCD.
89
What is the most common underlying etiology for aplastic anemia?
Idiopathic autoimmune suppression of hematopoiesis.
90
What are common historical findings associated with aplastic anemia?
* Benzene exposure
* Chemotherapy
* Hepatitis
* Radiation Exposure
* Pregnancy
91
A 45-year old patient that has underwent recent chemotherapy for their lung cancer presents for routine labs. Their CBC reveals that they have decreased RBCs, WBCs, and platelets. Labs taken annually reveal consistently low values as well. What has most likely occurred to their bone marrow?
Hypoplasia of their bone marrow, resulting in aplastic anemia, which is characterized by a decrease in all hematopoietic stem cell lines.
92
A 45-year old patient that has underwent recent chemotherapy for their lung cancer presents for routine labs. Their CBC reveals that they have decreased RBCs, WBCs, and platelets. Labs taken annually reveal consistently low values as well. What diagnostic study should be ordered to confirm the diagnosis of aplastic anemia?
Bone marrow biopsy showing hypocellular aspirate with little to no hematopoietic precursors.
93
A patient presents with decreased RBCs, WBCs, and platelet counts needs treatment. What medication is indicated for their underlying condition as first-line treatment?
Eltrombopag/Promacta, which is a multi lineage bone marrow growth factor.
| Aplastic anemia
94
What transfusion type is generally avoided in a patient presenting with aplastic anemia? Why?
WBC transfusion, due to risk of an immune reaction.
95
A 35-year old patient with an ANC of 250, platelets of 10,000, and absolute relic count of 20,000 presents to the ER. Besides transfusions, what medications/treatment are indicated for the management of their condition?
* Bone marrow transplant with a fully HLA-matched sibling donor (if pt < 40)
* Immunosuppression if transplant not viable.
* Equine ATG + cyclosporine + corticosteroids +/- eltrombopag or another bone marrow growth factor.
| Severe aplastic anemia.
96
What are the two bone marrow growth factors that are specifically to increase RBC production?
* Epoetin alfa
* Darbepoetin alfa (3x half-life)
| Both are Human EPO made via recombinant DNA.
97
What are the BBW associated with EPO treatment?
Increases risk of death due to MI, embolisms, thromboses, stroke.
| Increased RBC production = more blood
98
What primary SEs should a patient be counseled on when undergoing EPO treatment?
HTN and thrombosis are highly likely.
99
What hallmark finding suggests sideroblastic anemia?
Ring sideroblasts in bone marrow aspirates (blue ring)
| Requires a prussian blue stain.
100
What is the underlying etiology of sideroblastic anemia?
Reduced ability to synthesize heme due to impaired ability to incorporate iron into protoporphyrin IX.
101
What is the most common inheritance pattern of sideroblastic anemia?
X-linked.
102
What risk factors would make sideroblastic anemia more likely?
* Presence of a general myelodysplastic syndrome
* Alcohol abuse
* Lead poisoning
* Copper deficiency
* Chronic infection/inflammation
* Antimicrobial use (isoniazid, linezolid, chloramphenicol)
103
What finding on peripheral smear might suggest sideroblastic anemia?
Basophilic stippling (many purplish dots in a RBC)
104
A male patient presents with generalized fatigue and weakness. PE shows that he has palmar creases. His CBC reveals a microcytic anemia. Iron studies further show normal TIBC, elevated serum Fe and ferritin. What further diagnostic study should be ordered to confirm the suspected diagnosis?
Bone marrow aspirate showing erythroid hyperplasia and Prussian blue stain showing ringed sideroblasts.
| Sideroblastic anemia
105
A male patient presents with generalized fatigue and weakness. PE shows that he has palmar creases. His CBC reveals a microcytic anemia. Iron studies further show normal TIBC, elevated serum Fe and ferritin. His father was noted to have similar symptoms and was successfully treated with a supplement. What supplement was he most likely treated with?
Most likely B6 (pyridoxine) or B1 (thiamine)
| Some subtypes of sideroblastic anemia can respond to these.
106
What is the most common cause of anemia worldwide and the treatment?
Iron deficiency, treated via ferrous sulfate 325mg TID on an empty stomach.
107
What are the hallmark findings associated mainly with iron deficiency anemia?
* Pica
* Plummer-Vinson Syndrome
* Angular chelitis and smooth tongue
* Koilonychia
108
How does MCV change with iron deficiency anemia?
Early: Normocytic
Late: Microcytic
109
What is the primary concern associated with IM iron, specifically IM Iron dextran?
Back in the old days, IV dextran was associated with cutaneous siderosis, which is black staining of the hips/buttocks due to the iron.
110
What is the underlying etiology of anemia due to chronic inflammation/infection?
Increased hepcidin production, which decreases iron absorption and availability.
111
What lab finding will be most helpful in differentiating between iron deficiency anemia and anemia of chronic inflammation/infection?
Ferritin should be increased/normal in anemia of chronic inflammation/infection. They have adequate iron present in their body, but simply cannot utilize it.
112
What is the main underlying etiology for anemia of CKD?
Failure to secrete adequate EPO by the kidneys.
113
Why is patient history important to determine a normocytic, normochromic anemia?
Anemia can be due to chronic conditions, such as anemia of CKD, which presents with normal CBC and normal iron studies.
114
What are the anemias of hypometabolic states and their associated physiologies?
Anemia of endocrine disorders: decreased EPO secretion
Anemia of chronic liver disease: shortened RBC survival w/ decreased EPO secretion
Anemia of starvation: decreased EPO secretion
115
When is epoietin or darbepoietin indicated mainly?
Anemias where EPO secretion is diminished.
My PANCE Decks
flashcards
Decks in class (5)
# Cards
