Hematology 1- Iron, Thalassemia, Chronic Dz, Sideroblastic, Lead 10/21/16 Flashcards
(229 cards)
1
Q
What is Ferritin?
A
Storage form of iron. Easily accessible and small.
2
Q
What is Transferrin?
A
Iron transport protein
3
Q
What is TIBC? What does it indirectly measure?
A
Total iron binding capacity. Measure of iron binding capacity (and indirect assessment of transferrin)
4
Q
What is SI (Serum Iron)?
A
Amount of transferrin saturated of Iron Fe2+
5
Q
What is % saturation and formula formula?
A
transferrin saturation SI/TIBC x 100
6
Q
What is Reticulocyte Index? (RI)
A
Direct measure of bone marrow function. If keeping up with RBC need.
7
Q
What is Hemosiderin? Can the body use it easily?
A
Complex iron stores in macrophages. Difficult for the body to use.
8
Q
HCT normal range?
A
36-45
9
Q
Hgb normal range?
A
12-15
10
Q
WBC normal range?
A
5000-10,000
11
Q
Platelet normal range?
A
150,000-400,000
12
Q
Normal body iron range?
A
3-4g
13
Q
Define Anisocytosis. Which lab value?
A
Different size RBCs. Low MCV.
14
Q
Define Poiklocytosis
A
Different shaped cells
15
Q
MCV normal range?
A
80-100
16
Q
MCHC normal range?
A
33-35
17
Q
Hypochromic from which value?
A
MCHC lower than 33.
18
Q
Average American iron intake?
A
10-15mg/day
19
Q
Percent of dietary iron absorbed?
A
10%
20
Q
Iron requirement for male and non-mensturating females?
A
1mg/day
21
Q
Iron requirement for mensturating females range?
A
3-4mg/day
22
Q
Iron intake for pregger female range?
A
2-5mg/day
23
Q
Normal body iron content range?
A
3-4g
24
Q
How much iron lost through daily exfoliation in gut?
A
1mg/day
25
How much iron loss due to menses? Menses length range?
1mg/day, x4-7 days
26
Entire iron loss from preggers, delivery, and nursing?
About 1000mg
27
How much Fe lost in bleeding per 100mL of whole blood? (Trauma, acute bleed, loss d/t surgery, etc)
50mg
28
How much Fe in 100mL of whole blood?
50mg
29
Who gets Iron Deficiency Anemia most often?
Women more than men. D/t mensturation and preggers.
30
What is #1 cause of Iron Deficiency Anemia until proven otherwise?
BLEEDING!
31
Iron Deficiency Anemia causes NIMBLE stands for?
```
N=Need
I=Intake
M=Malabsorption
B=Blood
L=Loss
E=Excessive donation
```
32
Iron Deficiency Anemia causes other than bleeding?
1. Deficient diet
2. Decreased absorption (Celiac dz, Zn deficiency)
3. Increased requirements (preggers, lactation)
4. Hemoglobinuria
5. Iron sequestration
33
3 stages of Iron Deficiency Anemia?
1. Depletion of iron stores without anemia
2. Anemia with normal RBC size (normal MCV)
3. Anemia with reduced RBC size (low MCV)
34
Signs and symptoms of Iron Deficiency Anemia?
Fatigue, dizzy/weak, HA, tachycardia, dyspnea on exertion, palpitations
Beeturia, pica (eating weird things), pagophagia (craving for ice), dysphagia d/t esophageal web), pallor, conjunctival and palm pallor, smooth tongue, brittle nails, kolionychia (nails spooning), cheiolosis (breakdown of skin in corner of mouth when severe)
35
Three signs/symptoms of severe anemia? Why?
Tachycardia, dyspnea on exertion, palpitations. Due to decreased oxygen carrying capacity.
36
In Iron Deficiency labs (Serum Ferritin, Serum Iron, TIBC, and % Sat) which are low and increased?
Serum Ferritin=low
Serum Iron=low
% Sat=low
TIBC=increased
MCV low in anemia w/reduced RBC size.
37
MCV and MCHC in initial Iron Deficiency Anemia?
Low MCV, low MCHC. Microcytic, hypochromic
38
MCV and MCHC in severe Iron Deficiency Anemia? Aniso or Poik?
Low MCV, low MCHC.
Progression to anisocytosis and poikilocytosis.
Aniso=target cells
Poik=pencil and cigar shapes cells
39
Essentials of Dx for Iron Deficiency Anemia
Iron deficiency present.
Serum Ferritin less than 12 in absence of scurvy
-OR-
Anemia and Serum Ferritin less than 30
40
How to prevent Iron Deficiency Anemia in pregnant women in deficient?
Low dose Iron 30mg/day PO if deficient
41
When to screen girls 12-18 and nonpreggers childbearing age women for Iron Deficiency Anemia?
Screen q5-10y, annual screening for women at risk
42
In Iron Deficiency Anemia a Serum Ferritin less than __ and in the absence of ______
Serum Ferritin less than 12 in absence of scurvy
43
In Iron Deficiency Anemia a PT with anemia and a Serum Ferritin less than __ has essential to dx?
Anemia and Serum Ferritin less than 30
44
What is Reticulocyte Index value in Iron Deficiency Anemia?
Normal at less than 2.5.
45
What is #1 think to do for treating Iron Deficiency Anemia?
Search for blood loss! Do stool guaiac or colonoscopy to check for occult blood.
46
What is the DOC for iron repletion in Iron Deficiency Anemia?
Ferrous Sulfate 325mg (65mg elemental iron) PO TID x1-2 months, then daily for 3-6 months
47
What will CBC be at 3 weeks, 2 months, and 6 months after starting tx for Iron Deficiency Anemia?
3 weeks=half way to normal
2 months=normal
6 months=normal (replace storage)
48
In Iron Deficiency Anemia if CBC is normal at 2 months why continue tx for 6 months?
To replete iron stores
49
What is #1 ADR or PO Iron?
Constipation
50
How often to check for Iron Deficiency Anemia after tx completed?
Once a year
51
What to avoid when taking PO Iron?
Avoid dairy, fiber, food, tea, tannins, phosphates, antacids. All bind iron and prevent absorption.
52
What helps PO Iron absorption?
Orange Juice! Drink OJ!
53
When to use IV iron for Iron Deficiency Anemia?
If they fail PO iron. Might have GI malabsorption from IBD or gastric bypass or might have continued blood loss that can't be stopped (hemodialysis).
54
If PT on dialysis and not being transfused regularly and has Iron Deficiency Anemia what to do?
Supplement with IV Iron
55
If PT on dialysis and *is* being transfused regularly and has Iron Deficiency Anemia what to do?
Don't supplement iron!
56
When and how to take PO Iron?
Empty stomach. Use straw to avoid teeth stain.
57
If PO Iron not tolerated BID or TID what to do?
Use low dose at 1-2x/day or slow absorption prep (SlowFe)
58
Most common ADRs of PO Iron?
Nausea, constipation, diarrhea, dark stool
59
If PT not responding to PO Iron for Iron Deficiency Anemia what to consider?
Incorrect dx, celiac dz, ongoing bleeding, non-compliance
60
When to refer Iron Deficiency Anemia to hematology
If H and H below 9 and below 27%
61
What are signs of Zinc Deficiency on hands and buttocks?
Rashes.
Buttocks=dry, scaly, eczematous skin. Can become infected with Candida albicans.
Hands=enlarged fingers, paronychia, bright erythema on terminal phalanges
62
What can Zinc Deficiency cause malabsorption of?
Iron
63
Thalassemia is a reduction of what? What does it do to hemoglobin synthesis?
Reduction in synthesis of alpha or beta globin chains. Causes reduced hemoglobin synthesis.
64
Is Thalassemia hereditary?
YES!
65
What happens to MCV and MCH in Thalassemia?
Low MCV, low MCH. Hypochromic and microcytic anemia.
66
Heme is made up of how many porphyrin and rings and iron molecules?
Heme=4 porphyrin rings, 4 iron molecules
67
What is normal adult Globin made up of?
HgA. 2 alpha and 2 betas chains.
68
Alpha globin on which chromosome, how many sites, and how many pairs? Total from parents?
Chromosome 16, 4 sites, 2 from each parent. 4 total.
69
Beta globin on which chromosome and how many pairs? Total from parents?
Chromosome 11, 2 sites, 1 from each parent.
70
Beta globin chains can be mimicked by what?
Delta chains or Gamma chains.
71
Can Alpha globin chains be mimicked?
No.
72
Hgb A normally made up of which globin chains? How many? What percent of normal adult circulation
2 alpha, 2 beta.
| 98% of normal adult circulation.
73
Fetal hemoglobin is called what, made up of which globin chains, and does what to oxygen? Wears off when?
Hgb F. 2 alpha, 2 gamma.
| Very high affinity to oxygen, steals oxygen from mother. Wears off around 6 months.
74
Hgb H is made up of which and how many globin chains?
4 Beta chains
75
3 types of Thalassemia and transfusions?
1. Trait= lab findings, no clinical impact
2. Intermedia= moderate clinical impact, occasional transfusion
3. Major= Life threatening, transfusion dependent
76
Thalassemia iron study lab trends (SF, SI, TIBC, % Sat)?
SF=norm or inc
SI=norm or inc
% Sat=norm or ince
TIBC=NORMAL
77
Thalassemia RBC, MCV, MCH, Reticulocyte Count?
RBC=norm or elevated
MCV=LOW (lower than iron deficinecy)
MCH=low
Reticulocyte Count=elevated, above 2.5
78
What are Heinz Bodies (found in Thalassemia)?
Denatured hemoglobin found inside RBCs
79
Are Nucleated RBCs (NRBCs) found with Thalassemia?
Yup, you bechya.
80
Thalassemia essentials of Dx
1. Microcytosis disproportionate to degree of anemia
2. + fam history
3. Norm or elevated RBC count
4. Abnormal RBC morphology with microcytes, hypochromia, acanthocytes, and target cells
81
What are acanthocytes?
Spiked/spurred RBCs
82
Which 2 hemoglobins elevated in Beta-Thalassemia?
1. Hgb A2
| 2. Hgb F
83
Alpha-Thalassemia epi?
Southeast Asia and China
84
Genetic eti of Alpha-Thalassemia?
Gene deletions from Chromosome 16.
85
Each Alpha-Thalassemia gene deletion causes reduced alpha-globin chain synthesis by how much?
1/4
86
If missing two Alpha-Thalassemia sites on Chromosome 16 how much alpha-globin reduced by?
50%
87
Can alpha chains be mimicked or replaced by other chains?
No! Beta chains can, but alpha aren't beta chains.
88
What is the genotype of normal and silent carrier Alpha-Thalassemia?
Normal= aa/aa
| Silent carrier= aa/a-
89
What are the MCV and hematocrit or normal and silent carrier Alpha-Thalassemia?
Normal MCV, normal crit
90
What are genotypes for Alpha-Thalassemia Minor?
aa/-- or a-/a-
91
What are MCV and hematocrit for Alpha-Thalassemia Minor
MCV=60-75
| Crit=28-40%
92
What is genotype for Alpha-Thalassemia Hemoglobin H Dz?
a-/--
93
What are MCV and hematocrit for Alpha-Thalassemia Minor Hemoglobin H Dz?
MCV=60-75
| Crit=22-32%
94
What is genotype for Alpha-Thalassemia Hydrops Fetalis?
MCV=less than 60
| Crit=less than 18%
95
In Alpha-Thalassemia-1-Trait what is genetic eti and epi? Heterozygous or homozygous?
Alpha deletion heterozygous. a-/a-, each parent missing one site from Chromomsome 16.
Asian descent.
96
In Alpha-Thalassemia-2-Trait what is genetic eti and epi? Heterozygous or homozygous?
Alpha deletion homozugous. aa/--, one parent missing two sites from Chromomsome 16.
Black descent.
97
Life expectancy of Alpha-Thalassemia-Trait? Treatment?
Normal life expectancy. No treatment, just need to let them know.
98
What severity of anemia does Alpha-Thalassemia-Trait have?
Mild microcytic anemia.
99
What is the genetic eti of Alpha Thalassemia – Hgb H Disease? (Hint: How many sites missing?)
a-/--. 3 sites missing. Alpha deletion is heterozygous.
100
What type of anemia in Alpha Thalassemia – Hgb H Disease?
Chronic hemolytic anemia
101
What is the variability of severity in Alpha Thalassemia – Hgb H Disease?
Variable from Thalassemia minor to Thalassemia intermedia.
102
What are the signs and symptoms of Alpha Thalassemia – Hgb H Disease? PE findings?
Fatigue and weakness.
| PE: Pallor and splenomegaly
103
What severity of anemia in Alpha Thalassemia – Hgb H Disease?
Moderate anemia. HCT 22-32 (36-45 norm).
104
RBC in Alpha Thalassemia – Hgb H Disease?
Normal or increased
105
Reticulocyte Count in Alpha Thalassemia – Hgb H Disease?
Elevated
106
Does does Peripheral Smear show for Alpha Thalassemia – Hgb H Disease?
Markedly abnormal, microcytes, target cells, poikliocytosis
107
What does hemoglobin electrophoresis show on Alpha Thalassemia – Hgb H Disease?
10-40% Hgb H
108
What is MCV in Alpha Thalassemia – Hgb H Disease?
Low at 60-70. (80-100 norm)
109
Tx for Alpha Thalassemia – Hgb H Disease?
Folic Acid 1mg PO daily. Usually not transfusion dependent.
110
What to avoid with Alpha Thalassemia – Hgb H Disease?
Avoid medicinal iron and oxidative drugs like Sulfonamides which destabilize hemoglobin.
111
Tx if iron overloaded?
Iron chelation
112
Is Alpha Thalassemia – Hgb H Disease transfusion dependent? When?
Usually not. Only for hemolytic exacerbation like infx or stress or aplastic crisis.
113
Where is iron in Alpha Thalassemia – Hgb H Disease?
Still in system but not bound and becomes overloaded.
114
What is genetic eti of Alpha-thalassemia - Hydrops Fetalis?
--/--. All 4 sites missing. Alpha deletion heterozygous.
115
How much alpha globin is made in Alpha-thalassemia - Hydrops Fetalis?
None.
116
What happens to baby with Alpha-thalassemia - Hydrops Fetalis?
Stillborn. Incompatible with extra-uterine life.
117
What is HCT and MCV os Alpha-thalassemia - Hydrops Fetalis?
Hct=less than 18% (36-45)
| MCV=less than 60 (80-100)
118
Tx for Alpha-thalassemia - Hydrops Fetalis?
Fetal diagnosis with transfusion and/or parental stem cell infusion followed by allogenic bone marrow transplant has led to some survival.
119
Epi of Beta-thalassemia?
Major Mediterranean origin (Italian, Greek). Lesser Asian and Black population.
120
What is reduced in Beta-thalassemia?
Reduced Beta-globin chain synthesis.
121
Which chromosome is Beta globin gene located on? How many?
2 genes, 1 from each parent. Chromosome 11.
122
Can Beta globin be mimicked?
YES, by gamma and delta chains.
123
What is the type genetic mutation in Beta-thalassemia?
Point gene mutation, not gene deletion.
124
Children with Beta-thalassemia look like what until 6 months?
Normal at first, severe anemia after 6 months d/t Hgb F ceasing production and needing Beta chains.
125
In Beta-thalassemia what does Beta+ mean?
Reduced (not absent) synthesis of beta chains
126
In Beta-thalassemia what does Beta0 mean?
Absent beta chain expression
127
What are the two Beta-like globins?
Gamma and Delta
128
HemF and oxygen delivery to tissues?
HemF has high affinity for oxygen and doesn't easily release oxygen to tissues
129
What are the two types of Hgb in Beta-thalassemia?
Hgb A2 or Hgb F
130
Beta-thalassemia and RBC morphology?
Abnormal RBC morphology w/microcytes, hypochromia, acanthocytes
131
Beta-thalassemia should be considered if....?
Low MCV, normal ferritin, family hx
132
What is genetic eti of Beta-thalassemia-Minor?
Beta/Beta+ or Beta/beta0. Heterozygous.
133
What degree of anemia in Beta-thalassemia-Minor?
Clinically insignificant microcytic anemia
134
MCV in Beta-thalassemia-Minor?
Low 55-75 (80-100)
135
Hct in Beta-thalassemia-Minor?
Modest anemia 28-40 (36-45)
136
RBC in Beta-thalassemia-Minor?
RBC=Normal or increased
137
Reticulocyte Count in Beta-thalassemia-Minor?
Normal to slight increase (but poor product)
138
Peripheral smear in Beta-thalassemia-Minor?
Mild abnormalities: microcytes, target cells, basophillic stippling
139
What does Hemoglobin electrophoresis show in Beta-thalassemia-Minor?
Elevated Hgb A2 at 4-8%. Occasional Hgb F at 1-5%.
140
Tx for Beta-thalassemia-Minor?
No transfusions or tx needed. Identify so they don't go through repeat blood tests over and over. Refer to genetic counseling.
141
Genetic eti of Beta-thalassemia-Intermedia?
Beta+/Beta+. Homozygous but allows for higher rate of beta globin synthesis.
142
PE of Beta-thalassemia-Intermedia?
- Bony deformity d/t RBC production outside bone marrow
- -Facial abnormality, pathological fx
- Hepatosplenomegaly
- Norm at birth, severe anemia at 6months
143
HCT in Beta-thalassemia-Intermedia?
17-33% (36-45)
144
MCV in Beta-thalassemia-Intermedia?
Low 55-75 (80-100)
145
Reticulocyte Count in Beta-thalassemia-Intermedia?
Elevated
146
RBC morphology on peripheral smear in Beta-thalassemia-Intermedia?
Hypochromia, microcytosis, basophillic stippling, target cells
147
Hemoglobin electrophoresis in Beta-thalassemia-Intermedia?
Hgb A up to 30%, Hgb A2 up to 10%, HgbF 6-100%
148
Tx for Beta-thalassemia-Intermedia?
Regular transfusions not necessary, only occasionally. Refer to genetic counseling.
149
Survival for Beta-thalassemia-Intermedia?
Survive in adulthood and do well.
150
What type of anemia in Beta-thalassemia-Intermedia?
Chronic hemolytic anemia
151
Beta-thalassemia-Major aka?
Cooley anemia
152
Beta-thalassemia-Major genetic eti?
Beta0/Beta0 or Beta+/Beta+. Homozygous. Two abnormal sites or no sites at all.
153
When is Beta-thalassemia-Major diagnosed?
6-12 months of age
154
Beta-thalassemia-Major birth to 6 months?
Normal at birth, severe anemia at 6 months.
155
What type of RBC production in Beta-thalassemia-Major?
Extramedullary Hematopoiesis=RBCs being made outside bone marrow
156
PE of Beta-thalassemia-Major?
- Stunted growth, bone deformity (face deformed, pathologic fx)
- Hepatosplenomegaly
- Jaundice w/gallstones or hepatitis related cirrhosis
- Thrombophillia
157
Other complications with Beta-thalassemia-Major
So many.....liver, lungs, bones, heart, diabetes, etc
158
RBC and indicies with Beta-thalassemia-Major?
High red count, low indicies, large amount of odd looking cells
159
HCT and MCV in Beta-thalassemia-Major?
HCT=less than 10 (36-45) severe anemia
| MCV=LOW
160
Peripheral smear with Beta-thalassemia-Major?
Bizarre looking cells, severe poikilocytosis (diff shaped cells), hypochromia, microcytosis, target cells, basophillic sippling, NRBC
161
Hemoglobin electrophoresis with Beta-thalassemia-Major?
Little or no HgbA, variable Hgb A2, predominant Hgb F
162
TOC for Beta-thalassemia-Major?
-TOC=Allogenic stem cell transplant (only cure available)
163
Blood transfusions and other tx for Beta-thalassemia-Major?
Yes. Transfuse PRBC to prevent endogenous hematopoiesis.
- Maintain Hgb 9-10
- Iron Chelation if regular transfusions
- Folic Acid 1mg PO daily
- Splenectomy if indicated
164
When considering Thalassemia which tests to start with?
Start with CBC. If MCV/MCH low than consider Thalassemia.
165
Retic count is low in which type of Thalassemia?
Beta-Thalassemia-Major
166
HgbF at 90-100% in which type of Thalassemia?
Beta-Thalassemia-Major
167
Bone deformities in homozygous Beta-Thalassemia due to what?
Extramedullary hematopoiesis
168
Essentials of Thalassemia diagnosis?
-Microcytosis disproportionate to degree of anemia
-Positive family history or lifelong personal history of microcytic anemia (Low MCV)
-Normal or elevated RBC count
-Abnormal RBC morphology with microcytes, hypochromia, acanthocytes,
and target cells
169
Complications of chronic transfusions?
Transfusional hemosiderosis=iron overload. Builds up in liver and heart. Chelate!
170
When to refer Thalassemia?
- Severe to hematologist
- Minor or intermediate to genetic counselor
- Any PT with unexplained microcytic anemia (MCV less than 80)
171
What is the "Vampire" disease?
Porphyria
172
Porphyria eti?
Altered activities of enzymes within heme biosynthetic pathway. Can't produce porphyrin ring.
173
Porphyria signs and symptoms?
Anemia, ver pale skin, photosensitive (blisters in sun), dental abnormalities (pointed teeth)
174
Anemia of Chronic Dz labs? (Hint: 5)
CBC=mild anemia, normochromic, normoctyic
RI=low to normal, under 2.5
ESR and CRP=elevated
Iron Studies:
SF=norm or inc
SI=low (inflammation), normal (organ failure and elderly)
TIBC=norm or low
% Sat=norm or low
175
Gold standard for Anemia of Chronic Dz? How often done?
Bone marrow biopsy w/iron staining. Rarely done!
176
Anemia of Chronic Dz signs and symptoms
- Hx of known chronic dz, exam findings related to it
| - Sx of typical anemla: fatigue, dizziness, weakness
177
3 Types of Anemia of Chronic Dz?
1. Anemia of inflammation
2. Anemia of Organ Failure
3. Anemia of Elderly
178
Anemia of Inflammation epi?
People with known chronic dz
179
Anemia of Inflammation iron etiology?
Reduced uptake of iron from gut into bone marrow. Reduced response to EPO.
180
What happens to RBCs in Anemia of Inflammation?
Shortened RBC survival and hemolytic (breakdown) of RBCs
181
What is the "inflammatory marker" in Anemia of Inflammation?
Hepcidin
182
Anemia of Inflammation seen in PTs who have what diseases?
Inflammatory disorder, infectious process, or malignancy.
| EX: IBD, RA, chronic infection, malignancy, severe trauma, critically ill patients
183
What do cytokines to in Anemia of Inflammation?
Decrease RBC production
184
Anemia of Inflammation workup?
CBC, RI, Iron Studies
185
What sort of anemia seem in Anemia of Inflammation?
Normocytic or microcytic anemia
186
Anemia of Inflammation and ESR and CRP?
Elevated
187
Which iron type can be high in acute phase of Anemia of Inflammation?
Ferratin
188
PTs with Anemia of Inflammation should be evaluted for which two possible coexistent issues?
Iron deficiency
| Folic acid deficiency
189
What is SI in Anemia of Inflammation?
Low
190
Anemia of Elderly is what type of diagnosis?
Dx of exclusion
191
Anemia of Elderly eti? (Hint: EPO and inflammation)
- Resistance to EPO
| - Decrease to EPO production, d/t low level infammation
192
Anemia of Elderly has underlying issue of what?
Underlying chronic dz
193
Anemia of Elderly serum iron levels?
Normal
194
Anemia of Elderly ESR or CRP levels?
Elevated
195
Anemia of Elderly presentation?
Fatigue, dizzy, weak. Sx and exam findings related to chronic dz.
196
Anemia of Organ Failure EPO and RBC mass?
- Reduced EPO production
| - RBC mass decreased
197
Anemia of Organ Failure associated with what?
Chronic inflammation
198
Anemia or Organ Failure can coexist with what?
Iron deficiency or folic acid deficiency
199
ESR and CRP are specific or non-specific inflammatory markers?
Non-specific
200
Serum Ferritin below what indicates coexistent iron deficiency?
Less than < 30ng/mL indicates coexistent iron deficiency
201
Low Serum Iron (SI) associated with which Anemia of Chronic Dz?
Inflammatory
202
Normal Serum Iron (SI) associated with which Anemia of Chronic Dz?
Organ failure and elderly
203
Tx for Anemia of Chronic Dz?
- PRBC if indicated
- Treat underlying dz
- Treat folic acid of iron deficiency
- Parenteral recombinant EPO
204
What to check in Anemia of Chronic Dz to know if treating correctly?
Check inflammatory markers
205
Anemia of Chronic Dz refer to nephrology or hematology if considering which therapy?
Parenteral recombinant EPO
206
What is Heme comprised of?
Porpheryn ring and iron.
207
Sideroblastic Anemia etiology?
Defect in heme synthesis. Ringed sideroblast around nucleus preventing iron from getting in.
208
2 types of Sideroblastic Anemia
Acquired and Congenital
209
Sideroblastic Anemia defect in what?
Defect in biosynthesis heme synthesis (common) or mitochondrial protein synthesis (less common)
210
Shape of sideroblast in Sideroblastic Anemia? Causing what?
Ringed sideroblast. Causing ineffective erythropoiesis.
211
Iron levels in Sideroblastic Anemia?
Iron overload!
212
Sideroblastic Anemia RBCs?
Hypochromic, microcytic, poiklocytosis
213
Sideroblastic Anemia associated with deficiency of what?
Cu
214
Drugs that can cause Sideroblastic Anemia?
INH (TB test), chloramphenicol, Linezolid
215
Sideroblastic Anemia signs and symptoms
- Fatigue, dizziness, pallor
| - Hepatosplenomegaly
216
Sideroblastic Anemia symptoms vary depending on what?
Depending on the cause
217
Cytic RBCs in Sideroblastic Anemia are this or this?
Microcytic or macrocytic
218
Sideroblastic Anemia and neutropenia due to what?
Cu deficiency
219
Sideroblastic Anemia iron studies?
SI=elevated
SF=elevated
TIBC=low
% Sat=elevated
220
Sideroblastic Anemia and lead poisoning will show what on smear?
Basophillic stippling
221
Diagnostic study for Sideroblastic Anemia?
Bone marrow biopsy. Shows ringed sideroblasts.
222
What are Pappenheimber Bodies in Sideroblastic Anemia?
Seen with excess iron
223
Where does iron ring occur in Sideroblastic Anemia?
In bone marrow!
224
Sideroblastic Anemia tx?
- Transfuse if needed
| - Chelate if needed
225
If Sideroblastic Anemia d/t INH?
B6 will fix
226
Splenectomy and Sideroblastic Anemia? Contraindicated when?
Contraindicated if congenital
227
Lead Toxicity etiology? (Hint: protoporphyrin)
Decreases heme synthesis. Inhibits iron binding to protoporphyrin ring and inhibits protoporphyrin synthesis.
228
Lead Toxicity peripheral blood smear almost always shows what?
Basophilic stippling
229
Lead Toxicity anemia (MCV, MCH)?
Hypochromic, normocytic or microcytic
