Hematology Flashcards

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1
Q

There are few physiologic disturbances until hemoglobin reaches ≤ _____.

A

7-8 g/dL

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2
Q

Some signs and symptoms of anemia include:

A

Pallor, loss of palmar crease pigmentation, irritability, fatigue, exertional dyspnea, orthopnea, pica (iron deficiency), tachycardia, palpitations, headache, vertigo

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3
Q

_____ is the most important lab clue for the cause of anemia.

A

Reticulocyte count

  • Can also examine a peripheral blood smear and…
  • *Urinalysis, direct Coombs test, stool guaiac
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4
Q

An acquired failure of the hematopoietic stem cells that results in pancytopenia is known as _____ anemia.

A

Aplastic

  • Most often idiopathic in developed countries
  • *May be induced by certain drugs or infections → hepatitis and mononucleosis
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5
Q

How are severe cases of aplastic anemia treated? Mild cases?

A
  1. Severe- stem cell transplantation

2. Mild- Supportive treatment with transfusion of PRBCs and platelets

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6
Q

When CBC shows hemoglobin decreased from baseline, with markedly increased reticulocytosis you are dealing with _____ anemia.

A

Hemolytic

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7
Q

Bacterial and viral infections in children with sickle cell anemia can precipitate rapid _____, with sudden-onset of jaundice and pallor

A

Hemolysis

*Other causes- TTP, HUS, giant hemangioma, artificial heart valves, sepsis, DIC, Sickle Cell Anemia/Crisis

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8
Q

How do you treat hemolytic anemia?

A

Treat underlying infection!

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9
Q

Sickle cell disease is an autosomal _____ (recessive/dominant) genetic disorder of HgbSS. Affects 0.15% of African Americans.

A

Recessive

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10
Q

Sickle cell trait confers some resistance to ______ (specific disease).

A

Malaria

  • individuals with trait may have episodic hematuria and inability to concentrate urine
  • *affects about 8% of African Americans
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11
Q

Sickle cell disease can lead to ______ anemia because sickled cells are destroyed by the spleen.

A

Hemolytic

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12
Q

_____ is one of the first signs of hemolytic anemia, seen around 6-9 months.

A

Dactylitis- digit swelling. Also have delayed growth and development.

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13
Q

Osteomyelitis is a common infxn associated with ____.

A

Sickle cell disease

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14
Q

Vasoocclusion in spleen & RBC pooling leading to acute splenomegaly and rapidly decreasing hemoglobin is known as ______.

A

Splenic sequestration crisis

*Can lead to splenic infarction and functional asplenia

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15
Q

Some triggers of a sickle cell crisis include:

A

Cold weather, hypoxia, infxn, dehydration, ETOH, pregnancy.

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16
Q

Acute chest syndrome, back, abdominal, and bone pain are associated with a ____ _____ _____.

A

Sickle cell crisis

  • Priapism is also common :(
  • *Those with the trait may suffer from: pulmonary HTN, CHF, symptoms of fatigue, SOB due to decreased O2 affinity of HgbS
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17
Q

How is sickle cell disease diagnosed?

A
  1. CBC with peripheral blood smear:

DECREASED hemoglobin
DECREASED hematocrit
INCREASED reticulocytes

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18
Q

Management of sickle cell disease:

A
  1. IV hydration and Oxygen 1st step!!! Then narcotics for adequate pain control (AVOID Meperidine–> high doses can lead to seizures)
  2. Hydroxyurea: reduces frequency of pain crisis
  3. Folic Acid: needed for RBC production and DNA synthesis
  4. RBC transfusion therapy- only in a severe crisis
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19
Q

Children with sickle cell disease should be immunized for:

*Hint- SHiN

A

S. penumo

H. influenzae type B

N meningococcus

*should also receive prophylactic PCN from 4 months- 6 years.

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20
Q

Von Willebrand disease, Hemophilia A, & Hemophilia B are examples of _______.

A

Clotting factor disorders

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21
Q

______ is the MC inherited bleeding disorder.

A

Von Willebrand Disease

*vWF is necessary to anchor platelets to the injured vessel wall

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22
Q

Treatment of von willebrand includes:

A

DDAVP!!!!

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23
Q

____ is the MC acquired coagulopathy.

A

Vitamin K deficiency

  • Many coag factors depend on Vitamin K
  • *Neonates should receive a dose at birth
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24
Q

Vitamin K deficiency often occurs because of malabsorption, seen especially in patients with _____ (particular disease state).

A

Cystic fibrosis

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25
Q

Overdose of ______ (certain drug) can cause severe Vitamin K deficiency.

A

Warfarin

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26
Q

Deficiencies in Vitamin K may be secondary to:

A

Poor diet, liver failure, malabsorption, malnutrition, and the use of some drugs (especially broad-spectrum abx)

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27
Q

Hemophilia A is a result of factor ___ deficiency.

A

8

  • X-linked recessive
  • *Prolonged PTT but PT is normal
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28
Q

Hemophilia B is due to a deficiency in factor ___.

A

9

  • Christmas disease
  • *Indistinguishable from Hemophilia A clinically
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29
Q

Heparin overdose antidote is ____.

A

Protamine

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30
Q

Warfarin overdose antidote is ____.

A

Vitamin K

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31
Q

The most common hereditary hypercoaguable state is ______. Causes an increase in DVTs and PEs, especially in young patients.

A

Factor V Leiden

*Management- for high risk you need indefinite anti-coagulation. For moderate risk then prophylaxis during high-risk procedures

32
Q

A reduced level of inhibitors of the coagulation cascade is seen with Protein ___ and ____ Deficiencies.

A

C & S

  • C&S are natural anti-coagulants
  • *Treatment- oral anticoagulation for life
33
Q

Antithrombin III can be depleted in what conditions?

A

Pregnancy, liver disease, nephrotic syndrome, DIC

*Treated with anticoagulants

34
Q

Hormonal Hypercoaguable states include-

A
  1. Pregnancy (especially in 3rd trimester and up to 6 weeks postpartum)
  2. OCPs (especially when smoking)
35
Q

____ is a disorder that manifests clinically as recurrent venous or arterial thrombosis and/or fetal loss. Often occurs in association with SLE or other autoimmune disorder.

A

Antiphospholipid Syndrome

36
Q

Signs and symptoms of antiphospholipid syndrome include:

A

o Recurrent arterial or venous thromboses: DVTs or PEs
o Autoimmune diseases
o Thromboses after surgical procedures
o 2nd trimester miscarriages/Pre-eclampsia
o Migraines
o Thrombocytopenia
o Valvular heart disease → heart murmurs
o Limb asymmetry
o Livedo reticularis →vascular condition characterized by purplish discoloration of the skin

37
Q

Indefinite ____ therapy is often used for individuals with antiphospholipid syndrome.

A

Warfarin

38
Q

Platelet counts less than 150,000, a common cause of abnormal bleeding defines _____.

A

Thrombocytopenia

  • TTP is an example–> Thrombotic Thrombocytopenic Purpura (also ITP, NATP, and TAR)
  • *HIT–> Heparin Induced thrombocytopenia (seen in hospitalized patients)
39
Q

Treatment for ITP is rarely indicated. If it is it involves:

A

Corticosteroids, IVIG, anti-D immunoglobulin

*Avoid NSAIDs

40
Q

____ is found primarily in children, particularly after infection with E. coli.

A

Hemolytic Uremic Syndrome (HUS)

  • pregnancy and estrogen use may precipitate it in adults
  • *treat in kids- conservative, fluids and electrolyte balance
  • **treat in adults- plasmapheresis
41
Q

Some clinical findings of TTP include:

A
  • jaundiced or pale
  • purpura, petechiae, pallor, abdominal pain, fever
  • CNS sx- wax and wane over mins
  • may have significant renal dz
  • possibly pancreatitis
42
Q

____ is a severe illness that alters the homeostasis between hemorrhage and thrombosis. There is activation of both coagulation (thrombin) and fibrinolysis (plasmin).

A

Desseminated Intravascular Coagulation (DIC)

43
Q

Acute and chronic conditions associated with DIC:

A

Sepsis, burns, tissue injury, obstetric complications, trauma, asphyxia, malignancy, cirrhosis, severe transfusion reactions

44
Q

Clinical findings with DIC…

A
  • Skin and mucous membrane bleeding (particularly at puncture/wound sites) and shock are more common
  • Thrombosis (commonly digital ischemia and gangrene) less often predominates
45
Q

______ is associated with unrestrained growth of leukocytes and leukocyte precursors in the tissues

A

Acute leukemia

  • 30-40% of childhood cancer
  • *Hallmark- pancytopenia with circulating blasts
46
Q

___ is the MC type of acute leukemia.

A

ALL- Acute Lymphoblastic Leukemia (80%)

*AML (Acute Myeloid Leukemia) is primarily a disease of adulthood

47
Q

ALL has a peak incidence around 2-5 years and is MC in ____ (boys/girls).

A

Boys

48
Q

____ (B/T) lineage is the more favorable type of ALL.

A

B

49
Q

Clinical findings of ALL include:

A
  • Fever
  • Pallor
  • Petechiae or ecchymoses
  • Gingival bleeding / epistaxis / menorrhagia may be the presenting complaint
  • Lethargy / malaise
  • Anorexia
  • Bone or joint pain
50
Q

ALL is confirmed with ____ on the peripheral smear.

A

Blasts, bone marrow aspirate, or both

*Bone marrow biopsy should be done urgently!!

51
Q

High risk of tumor lysis syndrome associate with ALL

A

-hyperuricemia, hyperphosphatemia, hyperkalemia

52
Q

Bone pain, often in the spine, ribs, or proximal long bones is associated with ______ (type of cancer).

A

Multiple Myeloma

53
Q

A HIGH Transferrin and HIGH TIBC level suggests ____ anemia.

A

Iron deficiency

*With low Ferritin

54
Q

A LOW Transferrin and a LOW TIBC is associated with _____.

A

Anemia of chronic disease

*With high Ferritin

55
Q

A microcytic anemia w/ normal or increased serum Fe or no response to Fe treatment is known as _____.

A

Thalassemia

56
Q

_____ is EPISODIC hemolytic anemia associated with sulfa drugs, fava beans, and infections.

A

G6PD deficiency

57
Q

HUS has a higher associated with kidney involvement than TTP and does not classically have fever or ______.

A

Neuro sx

58
Q

The 1st step in the workup of anemia is a ____ count.

A

Retic

  • High retic= brisk bone marrow response to hemolysis or blood loss
  • *Low retic= deficient RBC production
59
Q

Low retic count can be broken down to: Normocytic (80-100), Microcytic (<80), or Macrocytic (>100).

A
  1. Microcytic Anemia- Fe deficiency, lead, thalassemia, early anemia of chronic dz.
  2. Normocytic Anemia- Anemia of chronic disease!, early iron deficiency
  3. Macrocytic Anemia- B12 deficiency, Folate deficiency (also liver dz, ETOH, HYPOthyroidism)
60
Q

Celiac disease and Crohns affect the ileum (and absorption) and therefore can lead to malabsorption and _____ deficiency.

A

B12

61
Q

Autoimmune destruction/loss of gastric parietal cells that secrete Intrinsic Factor is the defining characteristic of _____ anemia.

A

Pernicious

62
Q

Clinical manifestations of B12 deficiency include:

A

Paresthesias, gait abnormalities, memory loss, and dementia

GI- anorexia, diarrhea, glossitis

63
Q

B12 defiency should be treated with _____. Watch for signs of HYPO____ with treatment.

A

IM B12

Watch for HYPOKALEMIA

64
Q

Common etiologies of iron deficiency anemia include:

A

Excessive menstruation, occult blood loss (colon cancer, parasitic hookworms)

65
Q

Pagophagia (ice craving), pica, angular cheilitis, and koilonychia (nail spooning) are associated with ____ anemia.

A

Iron deficiency

66
Q

Management of iron deficiency anemia includes:

A

Iron replacement (on empty stomach)

*Vitamin C increases Fe absorption

67
Q

Acquired sideroblastic anemia that is MC in children is known as _____.

A

Lead poisoning anemia

68
Q

Think THALASSEMIA if microcytic anemia with NORMAL/HIGH SERUM FE or no response to Fe treatment

A

fyi

69
Q

Heinz bodies are associated with ______ deficiency.

A

G6PD

70
Q

_____ is a lymphocyte neoplasm that is bimodal (peaks at 20 then again >50y) and associated with EBV.

A

Hodgkin’s Lymphoma

71
Q

Clinical manifestations of Hodgkin’s Lymphoma include:

A
  1. Painless lymphadenopathy. Alcohol may induce node pain!

2. Systemic “B” symptoms: fever, night sweats, and weight loss

72
Q

Reed-Sternberg cell is associated with a diagnosis of _____ (Hodgkin/Non Hodgkin) Lymphoma.

A

Hodgkin

*Also, mediastinal LAD on PET/CT scan

73
Q

How is Hodgkin Lymphoma treated?

A
  1. Stage I, II, and IIIA- radiation therapy
  2. Stage IIIB, IV- combo chemo
    * Highly curable- “Good” Lymphoma
74
Q

____ is MC in individuals >50y. Peripheral LAD!! (Hodgkin/Non Hodgkin)

A

Non Hodgkin

*RF- Increased age, Immunosuppression (HIV)

75
Q

An overproduction of all 3 Myeloid cell lines (primarily RBCs) is known as ______.

A

Polycythemia

  • Peaks 50-60y, MC in men
  • *Caused by JAK2 mutation
76
Q

Clinical manifestations of polycythemia include:

A

HA, dizziness, tinnitus, PRURITUS (esp after hot bath), fatigue and thrombosis

*PE: Splenomegaly, flushed face

**Due to increased RBC mass

77
Q

How is polycythemia managed?

A

Phlebotomy! –> Done until hematocrit <45%

Hydroxyurea