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.
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
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”
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)
5
Q
What is the role of erythropoietin
A
stimulate red blood cell (RBC) production
Synthesized in the kidneys
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)
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
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
9
Q
How long do Reticulocytes circulate in the blood stream
A
120 days
10
Q
What is heme broken down into
A
The heme portion is broken down into biliverdin for transport in the blood.
11
Q
What are the three granulocytes
A
Basophils
Eosinophils
Neutrophils
12
Q
What are the 2 Agranulocytes
A
Monocytes
Lymphocytes
13
Q
Most abundant WBC
A
Neutrophils
14
Q
How many lobes do neutrophils have
A
3-5 normally
Less than that means immature
More than that means older
15
Q
What do neutrophil bands mean
A
Infection
Lots of young neutrophils
16
Q
What do hypersegmented neutrophils suggest
A
Vitamin B12 or Folate Deficiency
17
Q
How many lobes do eosinophils have
A
Bilobed
18
Q
Role of eosinophils
A
Modulate acute hypersensitivity reactions
Defend against parasitic infections
Defend against intracellular bacteria
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
20
Q
What is the WBC thats increased in Chronic Myelogenous Leukemia
A
Basophils
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
22
Q
What are the role of Lymphoctyes
A
T cells and B cells and NK cells
23
Q
What is the normal MCV
A
80-100
This defines microcytic vs macro anemia
24
Q
What is anicocytosis
A
A LARGE variation in MCV of the RBCs
25
What is a reticulocyte
Reticulocytes – immature RBCs that usually stay in the bone marrow until mature and then enter the circulation
26
What # define anemia
Men: Hct < 41% or Hgb < 13.5 g/dL
Women: Hct < 36% or Hgb < 12 g/dL
27
What is the most common cause of anemia
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
28
What is the W/u of Anemia
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
29
A progressing microcytic anemia should prompt what investigation
CA UPO
30
A pt presents with tachycardia, paleness, splenomegaly, jaundice
Think anemia
What other three findings would you find?
- Cheilosis (usually due to vitamin B12 deficiency)
- Smooth tongue (“geographic tongue”)
- Brittle and/or spooning nails (koilonychia)
31
What is ferritin
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
32
What is transferrin
Plasma iron transport protein
33
What is hemosiderin
```
Hemosiderin
-Found predominantly in macrophages
—From phagocytosis of erythrocytes
-Not a readily available source of iron
-Pathological increases in iron overload states
```
34
What is Ferroportin
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’)
35
What is Hepcidin
Hepcidin
- Key negative regulator of intestinal iron absorption as well as macrophage iron release
- ‘closes the iron door’
36
What does serum iron measure
Serum Iron
| -measures circulating iron, most of which is bound to the transport protein transferrin
37
What does TIBC measure
Total Iron-Binding Capacity (TIBC)
| -Measures the blood’s capacity to bind iron with transferrin
38
What does TSAT measure
TSAT
-% Transferrin saturation =
(Serum Fe/TIBC) x 100
39
What comes in an Iron panel
Serum Iron, ferritin, Transferrin, TIBC< and TSAT
+sTfR
=sTfR/log
40
What is sTfR ?
Soluble transferrin receptor (sTfR)
-Circulating transferrin receptor or serum transferrin receptor
—Serum concentration directly proportional to erythropoietic rate
—Inversely proportional to tissue iron availability
41
What iron study is inversely proportional to tissue iron availability
Soluble transferrin receptor (sTfR)
42
High sTfR-ferritin index is a sign of…
iron deficiency due to increased erythropoietic drive and low iron stores
43
what will the reticulocyte count be in iron def anemia
LOW!
RBC need Iron to be made
44
If the reticulocytes are low
The serum iron is low
The ferritin in low
And the TIBC is high
Think
Iron deficiency
45
If the reticulocytes are low
The serum iron and TIBC are low
With a normal or high ferritin
Think
Increased sedimentation or C-reactive proteins
Think Anemia of chronic Dz
46
If the reticulocytes are low
And the serum iron, Normal TIBC, Normal Ferritin
Think?
Obtain hemoglobin studies
Think Alpha Thalasemmia trait
Or Beta thalassemia train
Or Hemoglobin E
or C syndromes or disorders
47
If the Reticulocytes are low and the serum iron, TIBC, and ferritin are all elevated
Think
Obtain bone marrow studies iwth iron stains
Think Sideroblastic anemias
48
If the Reticulocytes are increased in microcytic anemia
Think/?
Review blood smears
And look for hemolysis Dz
49
What is the most common cause of anemia world wide
Iron deficiency is the most common nutritional deficiency in the world
Anemia is the most common manifestation
50
What is the tx to Iron Def Anemia
Goal of therapy = repair Hgb deficit and replenish storage iron
Oral replacement – Treatment of choice
Ferrous sulfate
51
What drug interaction should be avoided in Iron Def Anemia
Levothyroxine
Also avoid coffee, dairy, and spinach
52
What vitamin increases Iron absorption
C
53
When should IV iron be given to Deficiency pts
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
54
When should Iron Def anemia recover after Tx
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
55
New-onset iron deficiency anemia in a male > 50 years old OR a postmenopausal female:
Consider!?
an occult gastrointestinal malignancy until proven otherwise!
Evaluate for potential GI blood loss with EGD and colonoscopy
56
WHat is the most frequent anemia of hospitalized pts
Anemia of chronic dz
57
What is the progression of Anemia of chronic Dz
initially normocytic and normochromic
That eventually becomes Hypochromic and microcytic
58
What are the mutuation of Alpha and Beta thalassemia
Alpha thalassemia – due primarily to gene deletions causing reduced alpha-globin chain synthesis
Beta thalassemia – point mutations rather than deletions of the beta chain
59
Define thalassemia intermedia
thalassemia intermedia when there is an occasional red blood cell transfusion requirement or other moderate clinical impact
60
Define thalassemia major
thalassemia major when the disorder is life-threatening and the patient is transfusion-dependent (α - Hemoglobin H/Hydrops Fetalis)
61
How does Alpha thalassemia effect beta chains
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
62
What are the hallmark features of Thalassemia
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
63
What is a Mentxer Index
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
64
Target cells in anemia indicate
Thalassemia
65
Acanthocytes and Codocytes indicate
Thalassemia
66
Define sideroblastic anemia
Inability of erythroblasts to incorporate iron into protoporphyrin
Bone marrow produces ringed sideroblasts instead of healthy RBCs
Presence of basophilic stippling
67
What are the causes of sideroblastic anemia
Rx: Alcohol (MC) , Isonazid, cyclosporine, chloramphenicol
And lead poisoning
Copper or B6 deficiencies
Chemo/Rads
inherited
68
You find microcyctic anemia and Dimorphism cells
Ringed Sideroblastic (Prussian blue)
Or Erythroid hyperplasia
Sideroblastic anemia
R/o inherited with genetic testing
69
What vitamin can help Sideroblastic anemia
```
Vit B6
(Pyridoxine)
```
70
On labs you see an increased level of free erythrocyte protoporphyrin
Think
Lead poisoning
71
If a lab shows hypersegmented neutrophils or macro ovalocytes
Think
Vit B12 or Folate deficiency
Megaloblastic anemia
72
What is the diff between macrocyte and megaloblastic
Megaloblastic has a nucleus
Macrocyclic is just large
73
How is MMA in Vit B12 vs Folate deficiency
In Vit B12 MMA is high
74
WHat is required for B12 absorption
Intrinsic Factor
Deficiency here = pernicious anemia
75
How does chrons Dz commonly present
Terminal illietus
Where you absorb B12
Which means Megalopbalstic anemia
76
Metformin, PPI, and H2 blockers all lead to what deficiency
Vitamin B12 deficiency
77
In a folate deficiency
| What major finding will you see
Homocysteine elevation
78
IF MMA and Homocsytine are elevated think what kind of anemia
Vit B12
79
Describe Pernicious Anemia
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)
80
If a pt has S/s of B12 deficiency without obvious cause
And they are not responsicve to B12 therapy
Think
Pernicious Anemia
81
What is the appropriate response to Vit B12 treatment
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
82
What is Vit B9
Folate
83
What is the role or Folate
Folate serves as a cofactor in DNA synthesis
| Interference with DNA synthesis affects all rapidly dividing cells
84
What are the causes of NONmegaloblastic anemia
Alcohol abuse
COPD
Hypothyroidism
Accelerated Erythropoesis
(Hemolytic anemia)
Hepatic Dz
Bone Marrow failure
85
What are the medications that cause nonmegaloblastic anemia
Methotrexate
Benzene
Purine agonist (acyclovir)
Pyridimine
Hydroxyurea
Retrovir
86
What are the common causes of normocytic anemia
Chronic inflammation
Malignancy
CKD
Hypothyroid
Chronic Hepatic Dz
Aplastic Anemia
Hemolytic Anemia
Acute Blood Loss
87
What is the primary maker of EPO
The Kidney
88
What stimulates Hepcidin
Synthesis stimulated by:
| increases in iron body stores, infection, inflammation, or malignancy
89
What inhibits Hepcidin production
Hypoxemia and Increased Erythropoeitic demand
90
What are the two places that make EPO
Liver and Kidney
91
What is the Tx approach to Anemia of Chronic Disease
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
92
What Rx cause Aplastic anemia
Carbamazepine and phenytoin Chloramphenicol and sulfas
NSAIDS
Anti thyroid rx
Gold
93
What is the hallmark of aplastic anemia
Pancytopenia
94
What is required for the Dx of aplasia anemia
Bone Bx
95
What is the tx for Aplastic anemia
Treatment:
-Eliminate offending agent
—Immunosuppressive agents may be helpful
-Supportive care
—Bleeding – platelet transfusion
—Anemia – blood transfusion
—Marrow stimulating agents
Bone marrow transplant
96
Howell Jolly Bodies, indicates what …
nuclear remnants within red cells that are ordinarily removed by the spleen
—Indicates asplenia or splenic hypofunction
97
What are the types of intrinsic hemolytic anemias
Defect in the RBC itself
(usually hereditary)
- Membrane defects – hereditary spherocytosis
- Oxidation vulnerability – G6PD
- Hemoglobinopathies – sickle cell disease, thalassemia
98
What are the types of Extrinsic Heemolytic Anemia
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
99
What is Hereditary spherocytosis
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
What is the classic triad of Hereditary spherocytosis
Classic triad of
—mild anemia
— intermittent jaundice
—splenomegaly
Gallstones also common due to hemolysis
—Bilirubin stones
101
Hereditary Spherocytosis is at risk to what virus
At risk for aplastic crisis
—Parvovirus B19
(fever, myalgias, pancytopenia)
102
A pt presents with fever, myalgias, and pancytopenia
Think what viral infection
Parvovirus B19
103
What test can rule out immune-mediated hemolysis
Coombs test
104
What are the confirmatory tests for Heriditary spherocytosis
Osmotic fragility test
And EMA binding
105
What is the Tx for Heriditary spherocytosis
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
Describe Hereditary elliptocytosis
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
What is the most prevalent human enzyme deficiency in the world
G6PD
108
What is the effect of G6PD deficiency
Deficiency in G6PD renders RBCs susceptible to oxidative stress
109
What are the medication of concern in a G6PD deficiency
Anti malarias
110
Heinz bodies are from what …
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
What are the triggers of oxidative stress that are important in G6PD deficiency
Antimalarias (e.g., Primaquine)
Antibiotics
- Sulfonamides
(e. g., sulfamethoxazole, sulfadiazine)
- Nitrofurantoin
- Quinolones
- Dapsone
Fava beans
Naphthalene (mothballs)
112
G6PD and NSDQ
Think
Nitrofurantoin
Sulfonamides
Dapsone
Quinolones
113
What are the S/s of hemolytic crisis
In the presence of oxidizing agent
- Hemoglobinuria (dark urine)
- Jaundice
- Extreme flank/back pain
114
If you see bite cells and Heinz bodies
Think
G6PD deficiency
115
How do we treat a hemolytic crisis
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
Describe Sickle Cell Dz
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
What is the mutation in sickle cell
Mutated Beta globin
118
When do Pt with sickle cell trait presents with s/s
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
What is the hallmark of sickle cell Dz
Vaso-occlusive crisis
```
MI
CVA
Avasc necrosis
Infarctions
Priapism
```
Chest pain
SOB
Hypoxemia
PE
Sepsis
Aplastic Crisis
120
What are the chronic manifestations of Sickle Cell Dz
Chronic pain from tissue infarction
Pulmonary hypertension
Chronic renal failure
Osteomyelitis
Gallstones
Heart failure
Leg ulcers
—Ulceration over the lower anterior tibia
121
Ulcerations over the lower anterior tibia
Is a sign of what underlying blood dz
SIckle cell
122
What is the tx approach to sickle cell dz
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
Increased LDH, Increased Bilirubin and decreased Haptoglobin
Think
Hemolysis
124
What does 1 unit of pRBC raise the Hgb by
1.0g/dl of the HbG and 3% of the Hct
125
What is the role of FFP
Indicated to replete coagulation factors in patients with active bleeding or high-risk for bleeding
- Liver failure
- Warfarin-induced overanticoagulation
- Disseminated intravascular coagulation
126
What is the role of cryoprecipitate
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
What type of bacterial infection is common with red blood cell transfusions
Gram neg infections
128
What type of bacteria is common in platelet transfusions
Gram positive
129
After recieving blood the pt has a FEVER, Rigors, TachyHR, and a 30 degree change in BP
Think
Transfusion-transmitted bacterial infections (TTBI)
130
What is primary hemostasis
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
What is secondary hemostasis
Serial, cascading activation of clotting factors at the site of endothelial injury -formation of a fibrin clot
132
What factors does Vitamin K work on
Synthesis of factors II, VII, IX, X, protein C and S, are vitamin K dependent
133
What is the role of vWF
Majority synthesized and secreted by vascular endothelial cells
Forms complex with factor VIII
In plasma, protects factor VIII from proteolysis
134
What are the two types of hemophilia
Two primary types
-Type A (more common)
Factor VIII deficiency
-Type B (“Christmas Disease”)
Factor IX deficiency
135
How does Hemophilia A present
Hemarthroses
136
What is hemophilic arthropathy
The chronic effects of repeated hemorrhage into the knees
Destruction of articular cartilage
Synovial hyperplasia
Reactive changes in bone and adjacent tissue
137
What is the tx approach to hemophilia A
Desmopressin
| Increases vWF to stabilize fx VIII
138
What medications should hemophiliacs avoid
Asprin and NSAIDs
139
What is Type 1 VWF Dz
Type 1
Most common, autosomal dominant
Partial quantitative defect
140
What is type 2 vWF Dz
Type 2
Various subtypes
Autosomal dominant/recessive
Qualitative defect
141
What is Type III vWF Dz
Type 3
Most severe, autosomal recessive
Severe/complete absence of vWF
142
Where is vWF made
Synthesized in endothelial cells and megakaryocytes
143
A pt that presents with a bleeding d/o yet PT and PTT are normal
Think
VWF dz
144
What is the treatment for vWF dz
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
What vitamin is protein C dependent on
VT.. K
146
What is the primary role of Protien C
Primary role of aPC is to inactivate coagulation factors Va and VIIIa
necessary for thrombin generation and factor X activation
147
What is the patho of warfarin Induced Skin Necrosis
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
A pt presents with demarcated areas of purpura and necrosis due to vascular occlusion following warfarin tx
Think
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
Warfarin induced skin necrosis is most common in pts with what deficiency
Protein C or S
150
What is the role of antithrombin
Natural anticoagulant
Inhibits thrombin (factor IIa), factor Xa, and other proteases in the coagulation cascade
151
What is a Fx V Leiden mutation
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
What is the common prothrombin gene mutation
G20210A point mutation in the prothrombin gene
Leads to increased concentration of prothrombin in the circulation
153
Describe Antiphospholipid Syndrome
```
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
A female with multiple miscarriages, think of what bleeding d/o
test for antiphospholipid syndrome
155
When should we suspect a thrombophlia
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
What is the initial Dx of choice for a VTE
CT pA
157
What is the Tx for VTE
Anticoagulant with LMWH or Unfrac Heparin
Bridge to Warfarin
Can also use Fx Xa inhibitor or Dabigatran
158
What is the safest anticoagulant for pts with a GFR less than 30
Warfarin
159
What is the tx duration of an unprovoked DVT
Extended life long
160
What is the tx duration for a pt with pro eked DVT
3 months
161
What is the duration of tx for a pt with Cancer ande DVT
Life long tx
162
How do you Dx A thalassemia
Electro hemp-phoresis
163
You see basophilic stippling think
Sideroblastic anemia
164
What is hemolytic Uremic syndrome
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
What are the major differences in Acute v. Chronic ITP
Acute ITP
Most common in children
Usually follows viral illness
Chronic ITP
Most common in adults
Associated with secondary causes
166
Look up Hemolytic Uremia Syndrome
167
Look at hemolytic anemias
168
Where is EPO made
In the kidneys
In pts with CKD EPO is reduced which can lead to anemias
169
Where is thrombopoetin made
In the liver
When pts have liver failure or liver Dz , then thrombopoeitn in hindered leading to platelet D/o
170
Where do unused broken down heme groups go in the body
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
Which leukocyte is assoc with allergic reactions AND chronic inflammation
Basophils
172
WHere do T cells mature
In the thymus
173
What is the role of platelets
A nucleated lens shaped cells that are Active in blood clotting
– Release chemicals that cause vascular spasm
174
W hai is Red Cell Distribution Width (RDW)
Estimates range of volume and sizes of RBCs
Can tell you in the size of red cells is uniform or widely different
175
What is the MCH and and MCHC
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
What is the normal Iron content of the body and how much is on RBCs
Normal iron content of the body ~ 3-4g
RBCs ~ 1800-2000mg
177
What are the S.s common to all anemias
Pallor, palpitations, weakness, dizziness, easy fatigability, other nonspecific complaints
178
What are two S/s highly specific to Iron Def Anemia
Pica (pagophagia), koilonychia
179
How does Iron Def anemia look on peripheral smear
Micro, Hyopochromic and anisocytosis
180
What are the ADE of Iron supplementation
Constipation, abdominal pain, nausea, heartburn, dark stools
181
If a pt has symptomatic or unexplained microcytic anemia with target cells
Think
Thalassemia
182
What is the method of detection for thalassemia
Electrophoresis
183
What is the Tx for severe Thalassemia
Chronic infusions
Iron Chelating agents
Folic Acid supplements
AVOID SULFONAMIDES
Severe cases may need marrow transplant
184
You hear Prussian blue staining
Think
Sideroblastic Anemia
185
If a VIt B12 def corrects with Schilling test
Then…
Think Pernicious anemia
If it doesnt correct think Illeal Dz
Smal Bowel overgrowth Bacteria
Fish tapeworm or Drug induced mal absorption
186
How does B12 get to the liver from the stomach
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
If a pt has B12 deficiency what should you test for
Anti-IF abs (pernicious anemia)
188
What is the recovery time of B12 deficiency if properly treated
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
What GI D/o are common to a folate deficiency
(e.g., tropical sprue and non tropical sprue, Crohn disease)
190
If you see megaloblastoid precursor cells think…
Nonmegaloblastic, Macrocytic Anemia
191
What are the three hemolytic anemias that are normocytic
- Hereditary spherocytosis
- Glucose-6-phosphate dehydrogenase (G6PD) deficiency
- Sickle cell disease
192
How is anemia caused in chronic dz
Anemia is caused by the direct and indirect inhibitory effects of inflammatory cytokines on erythrocyte production
193
If a pt has CKD and anemia with a GFR less than 30
Give what tx
Erythropoiesis-stimulating agents (ESA)
194
A pt has cancer and is actively going through chemo
Has anemia
Give what?
Erythropoiesis-stimulating agents (ESA)
195
What viral infections cause Aplastic anemia
EBV
Hepatitis
HIV
Herpes
196
What are the S/s of -any to-Enid
Thrombocytopenia – petechiae and easy bruising
Leukopenia - infection
197
Is G6PD X or Y linked
X linked
198
Pts that has G6PD cant reduce what substance
Hydro peroxide in the body
199
A pt presents with hemoglobinuria, jaundice, and extreme flank/ back pain
Think what deficiency
G6PD
200
What is the curative tx for sickle cell dz
Hematopoietic stem cell transplant
| Usually for children and adolescents
201
When a PRBCs used
Most commonly used to raise hemoglobin
Increases oxygen carrying capacity
- Chronic anemia
- Symptomatic anemia
- Acute bleeding (hemodynamically stable)
- Palliative care
202
What are the indications for platelets
Prophylactic transfusion
Prevent spontaneous bleeding (thrombocytopenia)
Threshold vary depending on patient and clinical scenario
Therapeutic transfusion
Active bleeding
Preparation for invasive procedure
203
in general 6 units of Platelets should raise the platelet count how much
30,000
204
What are the viral agents of Transfusion Viral Infections
Hep B and C
HIV
205
What is Fx II
Prothrombin
206
What are the major actions of the platelet phase of hemostasis
Adhesion
(VWF and GPIb)
Activation
(GPIIb-IIIa)
and Aggrgation
207
When thrombin is active what does it turn on next
Thrombin activates fribrinogen and converts it to fibrin
208
What are the three steps to clot formation
vascular phase
Platelet phase
Coag cascade
209
Heparin actively enhances what function
Antithrombin
210
What factors do protein C and S inactivate
Va and VIIIa
211
Where is the mutation in Hemophillia A
On the F8 gene on Chromosome 28
212
What is the most common inherited bleeding D/o
VWF dz
213
What is the life span of platelets
5-9 days
214
What stimulates platelet production
Thrombopoetin
215
What cell makes platelets
Megakaryocytes
216
If you find abnormal platelet count on CBC what should you R/o
Rule out pseudothrombocytopenia
- Giant platelets counted as WBCs
- Platelet clumping
217
What is the most common cause of immune thrombocytopenia
Primary immune thrombocytopenia
Antibodies bind platelets, marking them for destruction in the spleen
218
What are the common causes of secondary immune thrombocytopenia
Autoimmune like rheumatoid
Infections like HIV, Hep C, H. Pylori CMV
Medications or CA
219
Is splenomegaly common in ITP
No!
220
What are the clinical features of ITP
1st: Purapura/ Petechia
2nd: Bleeding from membranes
3rd: Bleeding from viscera
221
If a pt presents with severe thrombocytopenia and mucous membrane bleeding within 7-14 days of starting a new drug
Think
ITP!
222
If you get a CBC with isolated thrombocytopenia
Think
ITP
223
What must you do to Dx thrombocytopenia (Immune)
Bone Bx
224
What is the Tx approach to ITP
If no bleeding is present: monitor
If bleeding and platelets are below 50K :
Glucocorticoids
IVIG and Platelet transfusions
In severe cases: splenectomy
225
What is the onset of HIT
5-10 days following Heparin exposure
226
Why does HIT happen
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
If you think the pt has HIT
What is the next step
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
What is the Tx for HIT
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
If a pt has HIT how long should they be anticoagulant-ed for…
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
What are the two types of Thrombotic Microangiopathy
Thrombotic Thrombocytopenic Purpura (TTP)
Hemolytic Uremic Syndrome (HUS)
231
If a pt presents with thrombocytopenia and Micro Hemolytic anemia (MAHA)
Think of what two conditions
Thrombotic Thrombocytopenic Purpura (TTP)
Hemolytic Uremic Syndrome (HUS)
232
What is the key deficiency in a pt with TTP
Deficiency in ADAMTS13
233
What does ADAMTS13 do
von Willebrand factor cleaving protease (vWFCP)
234
A 40 y/o female presents with fatigue, dyspnea, petechiae and abd pain and tenderness
What should be High of the DDx
TTP
235
What is the Pentad of TTP
- Neurologic abnormalities
- Microangiopathic hemolytic anemia
- Fever
- Abnormal renal function
- Thrombocytopenia
236
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
TTP
237
What is the Tx for TTP
Plasma exchange
+ Glucocorticoids
And RITUXIMAB
238
What is the most common toxic for HUS
SHIGA E. Coli
239
A 10 y/o child presents with petechial rash, HTN , and oliguria
With bloody diarrhea, and dehydration
From a Shiga infection
Think
HUS
240
What is the tx for HUS
Fluid and electrolyte management, anti-hypertensives, transfusions (platelets, pRBCs)
Often inpatient management
241
What medications should kids with HUS avoid
ABX, Antimotility agents and NSAIDS
242
Define thrombophilia
Abnormalities in coagulation or natural anticoagulant molecules that increase the risk of venous thromboembolism (VTE)
243
A pt develops skin lesions 3-10 days following initiation of warfarin
Think
Warfarin induced skin necrosis
244
What is the test of choice to eval proximal DVT
Venus US
245
What is the tx for confirmed DVT
heparin or LMWH with bridge to warfarin
246
What are the benefits of using Fx Xa inhibitors for a DVT
Does not require a heparin/Lovenox bridge
- Rivaroxaban
- Apixaban
- Edoxaban
Benefits – No INR monitoring or daily injections
