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Senoir 21-2 > Hematology > Flashcards

Hematology Flashcards

1
Q

What is a hemoatologist

A

board-certified internist, or pediatrician who has completed additional years of training in hematology.

Focuses on direct patient care and diagnosing and managing hematologic disease.

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2
Q

What is hematopathology

A

the study of disease of the blood and bone marrow and of the organs and tissues that use blood cells to perform their physiologic functions.

-lymph nodes, the spleen, thymus, and other lymphoid tissue

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3
Q

What is Dyscrasia

A

Dyscrasia: a nonspecific term that refers to a disease or disorder, especially of the blood.

Literal meaning – “bad mixture”

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4
Q

What is Diathesis

A

Diathesis: a constitutional predisposition toward a particular state or condition and especially one that is abnormal or diseased.

Bleeding diathesis –refers to an inherited or acquired disorder affecting primary or secondary hemostasis (hemorrhagic diathesis, bleeding tendency, bleeding disorder)

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5
Q

What is the role of erythropoietin

A

stimulate red blood cell (RBC) production

Synthesized in the kidneys

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6
Q

What is the role of Thrombopoetin

A

stimulates platelet production
Synthesized primarily in the liver
(to a minor degree in the kidney, spleen, and other organs)

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7
Q

What is the role cytokines

A

stimulate WBC production

Colony-stimulating factors (CSF) and Interleukins

Cytokines secreted by macrophages, B lymphocytes, T lymphocytes, and mast cells, as well as endothelial cells, fibroblasts, and stromal cells

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8
Q

What makes up a Hgb unit

A

Hgb unit consists of 2 alpha (α) and 2 beta (β) chains

Each chain contains ferrous iron in a heme ring which binds reversibly to oxygen

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9
Q

How long do Reticulocytes circulate in the blood stream

A

120 days

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10
Q

What is heme broken down into

A

The heme portion is broken down into biliverdin for transport in the blood.

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11
Q

What are the three granulocytes

A

Basophils
Eosinophils
Neutrophils

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12
Q

What are the 2 Agranulocytes

A

Monocytes

Lymphocytes

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13
Q

Most abundant WBC

A

Neutrophils

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14
Q

How many lobes do neutrophils have

A

3-5 normally

Less than that means immature
More than that means older

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15
Q

What do neutrophil bands mean

A

Infection

Lots of young neutrophils

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16
Q

What do hypersegmented neutrophils suggest

A

Vitamin B12 or Folate Deficiency

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17
Q

How many lobes do eosinophils have

A

Bilobed

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18
Q

Role of eosinophils

A

Modulate acute hypersensitivity reactions
Defend against parasitic infections
Defend against intracellular bacteria

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19
Q

What is the role of Basophils

A

Least common of all of the white blood cells

account for <1% of the total WBCs circulating in the blood

Commonly involved in allergic reactions and chronic inflammation

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20
Q

What is the WBC thats increased in Chronic Myelogenous Leukemia

A

Basophils

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21
Q

What is the role of Monocytes

A

The main phagocytic cells in the body
Liver (Kupffer cells)
Brain (Microglia)

They are agranular kidney shaped

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22
Q

What are the role of Lymphoctyes

A

T cells and B cells and NK cells

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23
Q

What is the normal MCV

A

80-100

This defines microcytic vs macro anemia

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24
Q

What is anicocytosis

A

A LARGE variation in MCV of the RBCs

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25
Q

What is a reticulocyte

A

Reticulocytes – immature RBCs that usually stay in the bone marrow until mature and then enter the circulation

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26
Q

What # define anemia

A

Men: Hct < 41% or Hgb < 13.5 g/dL
Women: Hct < 36% or Hgb < 12 g/dL

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27
Q

What is the most common cause of anemia

A

The most common cause of anemia is iron deficiency

Bleeding is the most common cause of iron deficiency in adults

Poor diet may result in folic acid deficiency and contribute to iron deficiency

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28
Q

What is the W/u of Anemia

A

History and physical examination

Laboratory evaluation - Initial

  • CBC
  • Reticulocyte count
  • Peripheral blood smear
  • CMP
  • LDH, Haptoglobin, Bilirubin (indirect)

Later, based on findings

  • Nutritional studies
  • Iron, B12, Folate
  • Bone marrow examination
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29
Q

A progressing microcytic anemia should prompt what investigation

A

CA UPO

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30
Q

A pt presents with tachycardia, paleness, splenomegaly, jaundice

Think anemia

What other three findings would you find?

A
  • Cheilosis (usually due to vitamin B12 deficiency)
  • Smooth tongue (“geographic tongue”)
  • Brittle and/or spooning nails (koilonychia)
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31
Q

What is ferritin

A

Ferritin

  • Cellular storage protein for iron
  • Found in virtually all cells of the body and in tissue fluid
  • Serum ferritin concentration usually correlates with total-body iron stores
  • Except under conditions of inflammation
  • Ferritin is an acute phase reactant
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32
Q

What is transferrin

A

Plasma iron transport protein

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33
Q

What is hemosiderin

A 
Hemosiderin
-Found predominantly in macrophages
—From phagocytosis of erythrocytes
-Not a readily available source of iron
-Pathological increases in iron overload states
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34
Q

What is Ferroportin

A

Ferroportin (the ‘iron door’)

  • The cellular iron exporter
  • Facilitates transport of iron to apotransferrin in macrophages for delivery to erythroid progenitor cells in the bone marrow prepared to synthesize hemoglobin (‘iron recycling’)
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35
Q

What is Hepcidin

A

Hepcidin

  • Key negative regulator of intestinal iron absorption as well as macrophage iron release
  • ‘closes the iron door’
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36
Q

What does serum iron measure

A

Serum Iron

-measures circulating iron, most of which is bound to the transport protein transferrin

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37
Q

What does TIBC measure

A

Total Iron-Binding Capacity (TIBC)

-Measures the blood’s capacity to bind iron with transferrin

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38
Q

What does TSAT measure

A

TSAT
-% Transferrin saturation =
(Serum Fe/TIBC) x 100

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39
Q

What comes in an Iron panel

A

Serum Iron, ferritin, Transferrin, TIBC< and TSAT
+sTfR
=sTfR/log

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40
Q

What is sTfR ?

A

Soluble transferrin receptor (sTfR)
-Circulating transferrin receptor or serum transferrin receptor
—Serum concentration directly proportional to erythropoietic rate
—Inversely proportional to tissue iron availability

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41
Q

What iron study is inversely proportional to tissue iron availability

A

Soluble transferrin receptor (sTfR)

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42
Q

High sTfR-ferritin index is a sign of…

A

iron deficiency due to increased erythropoietic drive and low iron stores

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43
Q

what will the reticulocyte count be in iron def anemia

A

LOW!

RBC need Iron to be made

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44
Q

If the reticulocytes are low
The serum iron is low
The ferritin in low

And the TIBC is high

Think

A

Iron deficiency

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45
Q

If the reticulocytes are low

The serum iron and TIBC are low

With a normal or high ferritin

Think

A

Increased sedimentation or C-reactive proteins

Think Anemia of chronic Dz

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46
Q

If the reticulocytes are low
And the serum iron, Normal TIBC, Normal Ferritin

Think?

A

Obtain hemoglobin studies

Think Alpha Thalasemmia trait
Or Beta thalassemia train
Or Hemoglobin E
or C syndromes or disorders

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47
Q

If the Reticulocytes are low and the serum iron, TIBC, and ferritin are all elevated

Think

A

Obtain bone marrow studies iwth iron stains

Think Sideroblastic anemias

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48
Q

If the Reticulocytes are increased in microcytic anemia

Think/?

A

Review blood smears

And look for hemolysis Dz

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49
Q

What is the most common cause of anemia world wide

A

Iron deficiency is the most common nutritional deficiency in the world
Anemia is the most common manifestation

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50
Q

What is the tx to Iron Def Anemia

A

Goal of therapy = repair Hgb deficit and replenish storage iron

Oral replacement – Treatment of choice

Ferrous sulfate

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51
Q

What drug interaction should be avoided in Iron Def Anemia

A

Levothyroxine

Also avoid coffee, dairy, and spinach

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52
Q

What vitamin increases Iron absorption

A

C

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53
Q

When should IV iron be given to Deficiency pts

A

Reserved for the patient who:
-Remains intolerant to PO iron despite dosage modifications

  • Unable to meet iron requirements with PO therapy
  • Chronic uncontrollable bleeding, hemodialysis

-Iron malabsorption
Ex: surgical resection of the small intestine

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54
Q

When should Iron Def anemia recover after Tx

A

Return of the Hct level halfway toward normal within 3 weeks
-return to baseline after 2 months

Hemoglobin/Hematocrit values should correct by 6-8 weeks

Continue therapy for 3-6 months after restoration of values to replace iron stores

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55
Q

New-onset iron deficiency anemia in a male > 50 years old OR a postmenopausal female:
Consider!?

A

an occult gastrointestinal malignancy until proven otherwise!
Evaluate for potential GI blood loss with EGD and colonoscopy

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56
Q

WHat is the most frequent anemia of hospitalized pts

A

Anemia of chronic dz

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57
Q

What is the progression of Anemia of chronic Dz

A

initially normocytic and normochromic

That eventually becomes Hypochromic and microcytic

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58
Q

What are the mutuation of Alpha and Beta thalassemia

A

Alpha thalassemia – due primarily to gene deletions causing reduced alpha-globin chain synthesis

Beta thalassemia – point mutations rather than deletions of the beta chain

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59
Q

Define thalassemia intermedia

A

thalassemia intermedia when there is an occasional red blood cell transfusion requirement or other moderate clinical impact

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60
Q

Define thalassemia major

A

thalassemia major when the disorder is life-threatening and the patient is transfusion-dependent (α - Hemoglobin H/Hydrops Fetalis)

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61
Q

How does Alpha thalassemia effect beta chains

A

In the presence of reduced alpha chains, the excess beta chains are unstable and precipitate, leading to damage of red blood cell membranes.

This leads to both intramedullary and peripheral hemolysis

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62
Q

What are the hallmark features of Thalassemia

A

Microcytic, hypochromic anemia (low Hct and Hgb)

Normal to elevated RBC count
—Small RBCs (**low MCV)
—Pale RBCs (low Mean Corpuscular hemoglobin (MCH)

Normal to elevated reticulocyte count

More severe cases
—Elevated hemolysis labs – LDH, indirect bili,
Decreased haptoglobin

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63
Q

What is a Mentxer Index

A

Calculation that is helpful in differentiating between IDA and Beta Thalassemia trait

MCV (fL) divided by RBC count (millions/mm3)

Value of < 13 suggests Beta Thalassemia trait

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64
Q

Target cells in anemia indicate

A

Thalassemia

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65
Q

Acanthocytes and Codocytes indicate

A

Thalassemia

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66
Q

Define sideroblastic anemia

A

Inability of erythroblasts to incorporate iron into protoporphyrin

Bone marrow produces ringed sideroblasts instead of healthy RBCs

Presence of basophilic stippling

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67
Q

What are the causes of sideroblastic anemia

A

Rx: Alcohol (MC) , Isonazid, cyclosporine, chloramphenicol

And lead poisoning

Copper or B6 deficiencies

Chemo/Rads
inherited

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68
Q

You find microcyctic anemia and Dimorphism cells
Ringed Sideroblastic (Prussian blue)
Or Erythroid hyperplasia

A

Sideroblastic anemia

R/o inherited with genetic testing

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69
Q

What vitamin can help Sideroblastic anemia

A 
Vit B6
(Pyridoxine)
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70
Q

On labs you see an increased level of free erythrocyte protoporphyrin

Think

A

Lead poisoning

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71
Q

If a lab shows hypersegmented neutrophils or macro ovalocytes

Think

A

Vit B12 or Folate deficiency

Megaloblastic anemia

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72
Q

What is the diff between macrocyte and megaloblastic

A

Megaloblastic has a nucleus

Macrocyclic is just large

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73
Q

How is MMA in Vit B12 vs Folate deficiency

A

In Vit B12 MMA is high

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74
Q

WHat is required for B12 absorption

A

Intrinsic Factor

Deficiency here = pernicious anemia

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75
Q

How does chrons Dz commonly present

A

Terminal illietus

Where you absorb B12

Which means Megalopbalstic anemia

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76
Q

Metformin, PPI, and H2 blockers all lead to what deficiency

A

Vitamin B12 deficiency

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77
Q

In a folate deficiency

What major finding will you see

A

Homocysteine elevation

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78
Q

IF MMA and Homocsytine are elevated think what kind of anemia

A

Vit B12

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79
Q

Describe Pernicious Anemia

A

An autoimmune form of vitamin B12 deficiency

Antibodies destroy gastric parietal cells
Lack of parietal cells = lack of IF =B12 deficiency

Autoimmune disease tend to cluster – suspect with other autoimmune disease
(e.g., Graves disease, Addison disease, hypothyroidism)

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80
Q

If a pt has S/s of B12 deficiency without obvious cause
And they are not responsicve to B12 therapy

Think

A

Pernicious Anemia

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81
Q

What is the appropriate response to Vit B12 treatment

A

Erythropoiesis starts to normalize within 1-2 days

Hypersegmented neutrophils will usually disappear within 10-14 days

Neurologic S&S are reversible if less than 6 months, but possibly permanent (areflexia) with longer durations

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82
Q

What is Vit B9

A

Folate

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83
Q

What is the role or Folate

A

Folate serves as a cofactor in DNA synthesis

Interference with DNA synthesis affects all rapidly dividing cells

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84
Q

What are the causes of NONmegaloblastic anemia

A

Alcohol abuse

COPD

Hypothyroidism

Accelerated Erythropoesis
(Hemolytic anemia)

Hepatic Dz

Bone Marrow failure

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85
Q

What are the medications that cause nonmegaloblastic anemia

A

Methotrexate

Benzene

Purine agonist (acyclovir)

Pyridimine

Hydroxyurea

Retrovir

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86
Q

What are the common causes of normocytic anemia

A

Chronic inflammation

Malignancy

CKD

Hypothyroid

Chronic Hepatic Dz

Aplastic Anemia

Hemolytic Anemia

Acute Blood Loss

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87
Q

What is the primary maker of EPO

A

The Kidney

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88
Q

What stimulates Hepcidin

A

Synthesis stimulated by:

increases in iron body stores, infection, inflammation, or malignancy

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89
Q

What inhibits Hepcidin production

A

Hypoxemia and Increased Erythropoeitic demand

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90
Q

What are the two places that make EPO

A

Liver and Kidney

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91
Q

What is the Tx approach to Anemia of Chronic Disease

A

Treat underlying condition

Erythropoiesis-stimulating agents (ESA)
—Cancer undergoing active
chemotherapy
—Chronic kidney disease - glomerular filtration rate (GFR) < 30 ml/min
—HIV treatments with evidence of myelosuppression

Iron supplements 
(generally not recommended if no iron deficiency)

Blood transfusion as necessary for severe or life-threatening anemia

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92
Q

What Rx cause Aplastic anemia

A

Carbamazepine and phenytoin Chloramphenicol and sulfas

NSAIDS

Anti thyroid rx

Gold

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93
Q

What is the hallmark of aplastic anemia

A

Pancytopenia

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94
Q

What is required for the Dx of aplasia anemia

A

Bone Bx

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95
Q

What is the tx for Aplastic anemia

A

Treatment:
-Eliminate offending agent
—Immunosuppressive agents may be helpful

-Supportive care
—Bleeding – platelet transfusion
—Anemia – blood transfusion
—Marrow stimulating agents

Bone marrow transplant

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96
Q

Howell Jolly Bodies, indicates what …

A

nuclear remnants within red cells that are ordinarily removed by the spleen

—Indicates asplenia or splenic hypofunction

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97
Q

What are the types of intrinsic hemolytic anemias

A

Defect in the RBC itself
(usually hereditary)

  • Membrane defects – hereditary spherocytosis
  • Oxidation vulnerability – G6PD
  • Hemoglobinopathies – sickle cell disease, thalassemia
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98
Q

What are the types of Extrinsic Heemolytic Anemia

A

External factor not caused by the RBC

Immune
—Autoimmune, drug toxicity

Microangiopathic
—Thrombotic thrombocytopenic purpura (TTP), hemolytic-uremic syndrome (HUS), cardiac valve hemolysis (mechanical)

Infection
—Malaria (plasmodium), clostridium, borrelia

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99
Q

What is Hereditary spherocytosis

A

Disorder caused genetic defect in proteins that make up the RBC cytoskeleton and plasma membrane

Loss of membrane surface area relative to intracellular volume 2/2 spherical shape
—Less deformable
—Osmotically fragile 
—Prone to hemolysis
—Splenic trapping

Unable to pass through splenic fenestrations

Phagocytosis by splenic macrophages

100
Q

What is the classic triad of Hereditary spherocytosis

A

Classic triad of
—mild anemia
— intermittent jaundice
—splenomegaly

Gallstones also common due to hemolysis
—Bilirubin stones

101
Q

Hereditary Spherocytosis is at risk to what virus

A

At risk for aplastic crisis
—Parvovirus B19
(fever, myalgias, pancytopenia)

102
Q

A pt presents with fever, myalgias, and pancytopenia

Think what viral infection

A

Parvovirus B19

103
Q

What test can rule out immune-mediated hemolysis

A

Coombs test

104
Q

What are the confirmatory tests for Heriditary spherocytosis

A

Osmotic fragility test

And EMA binding

105
Q

What is the Tx for Heriditary spherocytosis

A

Supportive depending on disease severity

—Folic acid supplementation

Transfusions (thresholds vary by age, severity, comorbidities, symptoms)

Splenectomy
—Typically reserved for severe HS (requiring regular transfusions)

106
Q

Describe Hereditary elliptocytosis

A

Characterized by the presence of elliptical or oval erythrocytes on the blood films of affected individuals

More prevalent in individuals of West African descent
—-elliptocytes may confer some resistance to malaria

Typically asymptomatic

107
Q

What is the most prevalent human enzyme deficiency in the world

A

G6PD

108
Q

What is the effect of G6PD deficiency

A

Deficiency in G6PD renders RBCs susceptible to oxidative stress

109
Q

What are the medication of concern in a G6PD deficiency

A

Anti malarias

110
Q

Heinz bodies are from what …

A

G6PD deficiency

Oxidize hemoglobin leads to denatured, leads to —precipitates (Heinz bodies)

Oxidative damage to RBC membrane , rigid and non-deformable , removal from circulation / cell lysis

111
Q

What are the triggers of oxidative stress that are important in G6PD deficiency

A

Antimalarias (e.g., Primaquine)

Antibiotics

  • Sulfonamides
    (e. g., sulfamethoxazole, sulfadiazine)
  • Nitrofurantoin
  • Quinolones
  • Dapsone

Fava beans

Naphthalene (mothballs)

112
Q

G6PD and NSDQ

Think

A

Nitrofurantoin
Sulfonamides
Dapsone
Quinolones

113
Q

What are the S/s of hemolytic crisis

A

In the presence of oxidizing agent

  • Hemoglobinuria (dark urine)
  • Jaundice
  • Extreme flank/back pain
114
Q

If you see bite cells and Heinz bodies

Think

A

G6PD deficiency

115
Q

How do we treat a hemolytic crisis

A

Avoidance of triggers
—Treat underlying infection
—Hydration
—Prevent nephrotoxic effects of hemoglobinuria

—Transfusion
—If Hb/Hct are dangerously low

Most hemolytic events are self-limiting

116
Q

Describe Sickle Cell Dz

A

Inherited disease
—Autosomal recessive disorder
1-mutated gene = Sickle cell trait
~300 million worldwide w/ trait

Point mutation on the β-globin gene
—Single amino acid replacement at codon 6
Glutamic acid -valine

Sickle hemoglobin (Hb S)

117
Q

What is the mutation in sickle cell

A

Mutated Beta globin

118
Q

When do Pt with sickle cell trait presents with s/s

A

Generally asymptomatic - amount of HbA in red cells of individuals with SCT are sufficient to prevent sickling except in the most unusual circumstances

Examples of exertional death have been found in pts with sickle cell trait

119
Q

What is the hallmark of sickle cell Dz

A

Vaso-occlusive crisis

MI
CVA 
Avasc necrosis 
Infarctions 
Priapism

Chest pain
SOB
Hypoxemia

PE

Sepsis
Aplastic Crisis

120
Q

What are the chronic manifestations of Sickle Cell Dz

A

Chronic pain from tissue infarction

Pulmonary hypertension

Chronic renal failure

Osteomyelitis

Gallstones

Heart failure

Leg ulcers
—Ulceration over the lower anterior tibia

121
Q

Ulcerations over the lower anterior tibia

Is a sign of what underlying blood dz

A

SIckle cell

122
Q

What is the tx approach to sickle cell dz

A

Treating anemia

  • Blood transfusions
  • Folic acid supplementation
Preventing pain crisis
-Lifestyle modifications
-Avoiding dehydration, high altitude, overly strenuous exercise
Hydroxyurea 
(increases amount of HbF)
—HbF has a potent anti-sickling effect

Managing pain crisis
-Hydration and analgesics
-Exchange transfusion
—Stoke, priapism, acute chest syndrome

123
Q

Increased LDH, Increased Bilirubin and decreased Haptoglobin

Think

A

Hemolysis

124
Q

What does 1 unit of pRBC raise the Hgb by

A

1.0g/dl of the HbG and 3% of the Hct

125
Q

What is the role of FFP

A

Indicated to replete coagulation factors in patients with active bleeding or high-risk for bleeding

  • Liver failure
  • Warfarin-induced overanticoagulation
  • Disseminated intravascular coagulation
126
Q

What is the role of cryoprecipitate

A

Derived from plasma

Contains:
Fibrinogen, factor VIII, factor XIII, von Willebrand Factor (vWF), fibronectin

Indicated for fibrinogen replacement
Not currently commonly used

-Acquired hypofibrinogenemia
cardiac surgery, liver transplant, postpartum hemorrhage, trauma/massive transfusion

127
Q

What type of bacterial infection is common with red blood cell transfusions

A

Gram neg infections

128
Q

What type of bacteria is common in platelet transfusions

A

Gram positive

129
Q

After recieving blood the pt has a FEVER, Rigors, TachyHR, and a 30 degree change in BP

Think

A

Transfusion-transmitted bacterial infections (TTBI)

130
Q

What is primary hemostasis

A

Platelets exposed to collagen, fibronectin, and other molecules in the subendothelial matrix at the site of vascular injury -platelet adherence, activation, and aggregation- initial platelet plug

131
Q

What is secondary hemostasis

A

Serial, cascading activation of clotting factors at the site of endothelial injury -formation of a fibrin clot

132
Q

What factors does Vitamin K work on

A

Synthesis of factors II, VII, IX, X, protein C and S, are vitamin K dependent

133
Q

What is the role of vWF

A

Majority synthesized and secreted by vascular endothelial cells

Forms complex with factor VIII

In plasma, protects factor VIII from proteolysis

134
Q

What are the two types of hemophilia

A

Two primary types
-Type A (more common)
Factor VIII deficiency

-Type B (“Christmas Disease”)
Factor IX deficiency

135
Q

How does Hemophilia A present

A

Hemarthroses

136
Q

What is hemophilic arthropathy

A

The chronic effects of repeated hemorrhage into the knees

Destruction of articular cartilage

Synovial hyperplasia

Reactive changes in bone and adjacent tissue

137
Q

What is the tx approach to hemophilia A

A

Desmopressin

Increases vWF to stabilize fx VIII

138
Q

What medications should hemophiliacs avoid

A

Asprin and NSAIDs

139
Q

What is Type 1 VWF Dz

A

Type 1
Most common, autosomal dominant

Partial quantitative defect

140
Q

What is type 2 vWF Dz

A

Type 2
Various subtypes
Autosomal dominant/recessive

Qualitative defect

141
Q

What is Type III vWF Dz

A

Type 3
Most severe, autosomal recessive

Severe/complete absence of vWF

142
Q

Where is vWF made

A

Synthesized in endothelial cells and megakaryocytes

143
Q

A pt that presents with a bleeding d/o yet PT and PTT are normal

Think

A

VWF dz

144
Q

What is the treatment for vWF dz

A

Mild bleeding
-Type 1, certain Type 2 subtypes
—Desmopressin (DDAVP)

Moderate to severe cases bleeding
Certain Type 2 subtypes and Type 3
—vWF concentrates and factor VIII

145
Q

What vitamin is protein C dependent on

A

VT.. K

146
Q

What is the primary role of Protien C

A

Primary role of aPC is to inactivate coagulation factors Va and VIIIa

necessary for thrombin generation and factor X activation

147
Q

What is the patho of warfarin Induced Skin Necrosis

A

Warfarin blocks the production of vitamin K dependent coagulation factors

Proteins C&S have shortest half life
-Depleted most rapidly; even more so in Protein C deficient patient

Leads to thrombus formation in the small vessels of the dermis and subcutaneous tissue

148
Q

A pt presents with demarcated areas of purpura and necrosis due to vascular occlusion following warfarin tx

Think

A

A pt with Protien C or S deficient that has developed warfarin induced skin necrosis

THink of this in a pt that just started Warfarin

149
Q

Warfarin induced skin necrosis is most common in pts with what deficiency

A

Protein C or S

150
Q

What is the role of antithrombin

A

Natural anticoagulant

Inhibits thrombin (factor IIa), factor Xa, and other proteases in the coagulation cascade

151
Q

What is a Fx V Leiden mutation

A

Hereditary thrombophilia

Mutation in the factor V gene
encodes the factor V protein in the coagulation cascade

FVL renders factor V insensitive to the actions of activated protein C

152
Q

What is the common prothrombin gene mutation

A

G20210A point mutation in the prothrombin gene

Leads to increased concentration of prothrombin in the circulation

153
Q

Describe Antiphospholipid Syndrome

A 
Disorder in which vascular thrombosis and/or pregnancy complications attributable to placental insufficiency occur in patients with laboratory evidence for antibodies!! directed against proteins that bind to phospholipids
Antiphospholipid antibodies (aPL)

May be primary disease or associated with SLE

Think of this in a pt with multiple miscarriages

154
Q

A female with multiple miscarriages, think of what bleeding d/o

A

test for antiphospholipid syndrome

155
Q

When should we suspect a thrombophlia

A

Known family history

Strong family history of VTE

First VTE event before age 50

Recurrent VTE

VTE in an unusual site such as portal, mesenteric, or cerebral vein

Warfarin induced skin necrosis (PC)

Multiple miscarriages

156
Q

What is the initial Dx of choice for a VTE

A

CT pA

157
Q

What is the Tx for VTE

A

Anticoagulant with LMWH or Unfrac Heparin

Bridge to Warfarin

Can also use Fx Xa inhibitor or Dabigatran

158
Q

What is the safest anticoagulant for pts with a GFR less than 30

A

Warfarin

159
Q

What is the tx duration of an unprovoked DVT

A

Extended life long

160
Q

What is the tx duration for a pt with pro eked DVT

A

3 months

161
Q

What is the duration of tx for a pt with Cancer ande DVT

A

Life long tx

162
Q

How do you Dx A thalassemia

A

Electro hemp-phoresis

163
Q

You see basophilic stippling think

A

Sideroblastic anemia

164
Q

What is hemolytic Uremic syndrome

A

Defined by the simultaneous occurrence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury

One of the main causes of AKI in children

Leads to oliguric or anuric renal failure

165
Q

What are the major differences in Acute v. Chronic ITP

A

Acute ITP
Most common in children
Usually follows viral illness

Chronic ITP
Most common in adults
Associated with secondary causes

166
Q

Look up Hemolytic Uremia Syndrome

A
167
Q

Look at hemolytic anemias

A
168
Q

Where is EPO made

A

In the kidneys

In pts with CKD EPO is reduced which can lead to anemias

169
Q

Where is thrombopoetin made

A

In the liver

When pts have liver failure or liver Dz , then thrombopoeitn in hindered leading to platelet D/o

170
Q

Where do unused broken down heme groups go in the body

A

Unused heme groups can be recycled and used in hematopoiesis, or can be converted into bilirubin and used to make bile in the liver.

Iron ions can also be transferred to the protein ferritin for storage in the liver.

171
Q

Which leukocyte is assoc with allergic reactions AND chronic inflammation

A

Basophils

172
Q

WHere do T cells mature

A

In the thymus

173
Q

What is the role of platelets

A

A nucleated lens shaped cells that are Active in blood clotting

– Release chemicals that cause vascular spasm

174
Q

W hai is Red Cell Distribution Width (RDW)

A

Estimates range of volume and sizes of RBCs

Can tell you in the size of red cells is uniform or widely different

175
Q

What is the MCH and and MCHC

A

Mean cell hemoglobin (MCH)
—The average quantity of hemoglobin in red blood cells

Mean cell hemoglobin concentration (MCHC)
—The average concentration of hemoglobin inside red blood cells (this takes the size/volume of the RBC into account)

176
Q

What is the normal Iron content of the body and how much is on RBCs

A

Normal iron content of the body ~ 3-4g

RBCs ~ 1800-2000mg

177
Q

What are the S.s common to all anemias

A

Pallor, palpitations, weakness, dizziness, easy fatigability, other nonspecific complaints

178
Q

What are two S/s highly specific to Iron Def Anemia

A

Pica (pagophagia), koilonychia

179
Q

How does Iron Def anemia look on peripheral smear

A

Micro, Hyopochromic and anisocytosis

180
Q

What are the ADE of Iron supplementation

A

Constipation, abdominal pain, nausea, heartburn, dark stools

181
Q

If a pt has symptomatic or unexplained microcytic anemia with target cells

Think

A

Thalassemia

182
Q

What is the method of detection for thalassemia

A

Electrophoresis

183
Q

What is the Tx for severe Thalassemia

A

Chronic infusions

Iron Chelating agents

Folic Acid supplements

AVOID SULFONAMIDES

Severe cases may need marrow transplant

184
Q

You hear Prussian blue staining

Think

A

Sideroblastic Anemia

185
Q

If a VIt B12 def corrects with Schilling test

Then…

A

Think Pernicious anemia

If it doesnt correct think Illeal Dz
Smal Bowel overgrowth Bacteria

Fish tapeworm or Drug induced mal absorption

186
Q

How does B12 get to the liver from the stomach

A

Acid in the stomach stimulates pepsin release which then separates B12 from food

Gastric Pareital cells release Intrinsic Fx that binds to B12 and allows it to be absorbed into the terminal Ileum

From there is carried in the plasma to the liver

187
Q

If a pt has B12 deficiency what should you test for

A

Anti-IF abs (pernicious anemia)

188
Q

What is the recovery time of B12 deficiency if properly treated

A

Erythropoiesis starts to normalize within 1-2 days

Hypersegmented neutrophils will usually disappear within 10-14 days

Neurologic S&S are reversible if less than 6 months, but possibly permanent (areflexia) with longer durations

189
Q

What GI D/o are common to a folate deficiency

A

(e.g., tropical sprue and non tropical sprue, Crohn disease)

190
Q

If you see megaloblastoid precursor cells think…

A

Nonmegaloblastic, Macrocytic Anemia

191
Q

What are the three hemolytic anemias that are normocytic

A
  • Hereditary spherocytosis
  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency
  • Sickle cell disease
192
Q

How is anemia caused in chronic dz

A

Anemia is caused by the direct and indirect inhibitory effects of inflammatory cytokines on erythrocyte production

193
Q

If a pt has CKD and anemia with a GFR less than 30

Give what tx

A

Erythropoiesis-stimulating agents (ESA)

194
Q

A pt has cancer and is actively going through chemo

Has anemia

Give what?

A

Erythropoiesis-stimulating agents (ESA)

195
Q

What viral infections cause Aplastic anemia

A

EBV

Hepatitis

HIV

Herpes

196
Q

What are the S/s of -any to-Enid

A

Thrombocytopenia – petechiae and easy bruising

Leukopenia - infection

197
Q

Is G6PD X or Y linked

A

X linked

198
Q

Pts that has G6PD cant reduce what substance

A

Hydro peroxide in the body

199
Q

A pt presents with hemoglobinuria, jaundice, and extreme flank/ back pain

Think what deficiency

A

G6PD

200
Q

What is the curative tx for sickle cell dz

A

Hematopoietic stem cell transplant

Usually for children and adolescents

201
Q

When a PRBCs used

A

Most commonly used to raise hemoglobin

Increases oxygen carrying capacity

  • Chronic anemia
  • Symptomatic anemia
  • Acute bleeding (hemodynamically stable)
  • Palliative care
202
Q

What are the indications for platelets

A

Prophylactic transfusion

Prevent spontaneous bleeding (thrombocytopenia)

Threshold vary depending on patient and clinical scenario

Therapeutic transfusion

Active bleeding

Preparation for invasive procedure

203
Q

in general 6 units of Platelets should raise the platelet count how much

A

30,000

204
Q

What are the viral agents of Transfusion Viral Infections

A

Hep B and C

HIV

205
Q

What is Fx II

A

Prothrombin

206
Q

What are the major actions of the platelet phase of hemostasis

A

Adhesion
(VWF and GPIb)

Activation
(GPIIb-IIIa)

and Aggrgation

207
Q

When thrombin is active what does it turn on next

A

Thrombin activates fribrinogen and converts it to fibrin

208
Q

What are the three steps to clot formation

A

vascular phase
Platelet phase
Coag cascade

209
Q

Heparin actively enhances what function

A

Antithrombin

210
Q

What factors do protein C and S inactivate

A

Va and VIIIa

211
Q

Where is the mutation in Hemophillia A

A

On the F8 gene on Chromosome 28

212
Q

What is the most common inherited bleeding D/o

A

VWF dz

213
Q

What is the life span of platelets

A

5-9 days

214
Q

What stimulates platelet production

A

Thrombopoetin

215
Q

What cell makes platelets

A

Megakaryocytes

216
Q

If you find abnormal platelet count on CBC what should you R/o

A

Rule out pseudothrombocytopenia

  • Giant platelets counted as WBCs
  • Platelet clumping
217
Q

What is the most common cause of immune thrombocytopenia

A

Primary immune thrombocytopenia

Antibodies bind platelets, marking them for destruction in the spleen

218
Q

What are the common causes of secondary immune thrombocytopenia

A

Autoimmune like rheumatoid

Infections like HIV, Hep C, H. Pylori CMV

Medications or CA

219
Q

Is splenomegaly common in ITP

A

No!

220
Q

What are the clinical features of ITP

A

1st: Purapura/ Petechia
2nd: Bleeding from membranes
3rd: Bleeding from viscera

221
Q

If a pt presents with severe thrombocytopenia and mucous membrane bleeding within 7-14 days of starting a new drug

Think

A

ITP!

222
Q

If you get a CBC with isolated thrombocytopenia

Think

A

ITP

223
Q

What must you do to Dx thrombocytopenia (Immune)

A

Bone Bx

224
Q

What is the Tx approach to ITP

A

If no bleeding is present: monitor

If bleeding and platelets are below 50K :
Glucocorticoids
IVIG and Platelet transfusions

In severe cases: splenectomy

225
Q

What is the onset of HIT

A

5-10 days following Heparin exposure

226
Q

Why does HIT happen

A

Formation of IgG antibody to heparin-platelet factor 4 (PF4) complexes

IgG and heparin-PF4 complexes binds to and activates the platelets leading to prothrombotic state

227
Q

If you think the pt has HIT

What is the next step

A

Once HIT is suspected, the clinician must establish the diagnosis by performing a screening PF4-heparin antibody enzyme-linked immunosorbent assay (ELISA)

Use the 4T score
(greater than 6 means very likely0

228
Q

What is the Tx for HIT

A

Discontinue all forms of heparin

Begin treatment with direct thrombin inhibitor, fondaparinux, or DOAC

Until platelet count has recovered to at least 100,000/µL

Warfarin is contraindicated as initial treatment of HIT because of its potential to transiently worsen hypercoagulability.

When platelet count at 100,000/µL, Warfarin may be initiated

R.o DVT

229
Q

If a pt has HIT how long should they be anticoagulant-ed for…

A

In patients with documented thrombosis, anticoagulation should continue for 3-6 months after the platelet count has recovered

In patients without documented thrombosis, anticoagulation should continue for 30 days after the platelet count has recovered

REFER ALL!!

230
Q

What are the two types of Thrombotic Microangiopathy

A

Thrombotic Thrombocytopenic Purpura (TTP)

Hemolytic Uremic Syndrome (HUS)

231
Q

If a pt presents with thrombocytopenia and Micro Hemolytic anemia (MAHA)

Think of what two conditions

A

Thrombotic Thrombocytopenic Purpura (TTP)

Hemolytic Uremic Syndrome (HUS)

232
Q

What is the key deficiency in a pt with TTP

A

Deficiency in ADAMTS13

233
Q

What does ADAMTS13 do

A

von Willebrand factor cleaving protease (vWFCP)

234
Q

A 40 y/o female presents with fatigue, dyspnea, petechiae and abd pain and tenderness

What should be High of the DDx

A

TTP

235
Q

What is the Pentad of TTP

A
  • Neurologic abnormalities
  • Microangiopathic hemolytic anemia
  • Fever
  • Abnormal renal function
  • Thrombocytopenia
236
Q

On peripheral smear you see Schistocytis

And on CBC you see NML PT and PTT

With evidence of hemolysis : ↑ LDH, ↑ indirect bilirubin, ↓ haptoglobin, ↑ reticulocyte count

Think

A

TTP

237
Q

What is the Tx for TTP

A

Plasma exchange

+ Glucocorticoids
And RITUXIMAB

238
Q

What is the most common toxic for HUS

A

SHIGA E. Coli

239
Q

A 10 y/o child presents with petechial rash, HTN , and oliguria

With bloody diarrhea, and dehydration

From a Shiga infection

Think

A

HUS

240
Q

What is the tx for HUS

A

Fluid and electrolyte management, anti-hypertensives, transfusions (platelets, pRBCs)

Often inpatient management

241
Q

What medications should kids with HUS avoid

A

ABX, Antimotility agents and NSAIDS

242
Q

Define thrombophilia

A

Abnormalities in coagulation or natural anticoagulant molecules that increase the risk of venous thromboembolism (VTE)

243
Q

A pt develops skin lesions 3-10 days following initiation of warfarin

Think

A

Warfarin induced skin necrosis

244
Q

What is the test of choice to eval proximal DVT

A

Venus US

245
Q

What is the tx for confirmed DVT

A

heparin or LMWH with bridge to warfarin

246
Q

What are the benefits of using Fx Xa inhibitors for a DVT

A

Does not require a heparin/Lovenox bridge

  • Rivaroxaban
  • Apixaban
  • Edoxaban

Benefits – No INR monitoring or daily injections

Senoir 21-2 (77 decks)

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