Hematopoietic Disorder

Irondeficiency anemia
Vit B12 Def & Folate Def Anemia
anemia
G-6-PD Deficiency
Thalassemia

Anemia
Definition
- A reduction in number of RBCs
World Health Organization defined
anemia as
Male : Hb < 13 g/dL (8.07 mmol/L)
Female : Hb < 12 g/dL (7.45 mmol/L)

Pathophysiology
Macrocyti
c
Morpholo
gy

Vit B12 def.

Folic acid
def.
Drug
induced

Deficiency

Etiology

Iron
Vitamin B12
Folic acid
Pyridoxine

Hypochromic
, microcytic
Iron def.
Genetic abn.
Sickle cell
anemia
Thalassemia

Central
impaired bone
marrow function
-ACD
-Anemia of
elderly
-Malignant bone
marrow dis.

Normocytic
anemias
Recent blood loss
Hemolysis
BM failure
ACD
Renal failure
Endrocrine dis.
Myeloplastic
anemias

Peripheral
Bleeding
(hemorrhage)
Hemolysis
(hemolytic
anemias)

Normal Hematologic Values

Test

Reference Range (18

49 yr)

Hgb (g/dL)

M 13.517.5; F 12.016.0

Hct (%)

M 41.053.0; F 35.046.0

MCV (m3)

80-100

MCHC (%)

31-37

MCH (pg)

28-34

RBC (106/mm3)

4.5-5.9

RDW (%)

11-16

Reticulocyte index (%)

1-2.5

Serum iron (g/dL)

TIBC(g/dL)

M 50160; F 40150
250-400

Ferritin (ng/mL)

M 15200; F 12150

Folate (ng/mL)

1.8-16

Vit B12 (pg/mL)

100-900

Erythropoietin (U/mL)

0.01-0.03

Clinical Presentations
Acute onset

Chronic anemia

Cardiorespiratory

Fatigue, headache,

symptoms

vertigo, faintness

-Tachycardia

Sensitive to cold, pallor

-Lightheadedness

Loss of skin tone

-Breathlessness
Goals
Alleviate signs and symptoms
Correct the underlying etiology
Prevent recurrent anemia

Irondeficiency anemia

koilonychia

Angular stomatitis and

glossitis

Treatment
1. Control of the underlying causes
2. Dietary supplement
3. Therapeutic iron preparation
200 mg Fe/day

Iron products
Salt

Elemental Iron (%)

Ferrous sulfate
Ferrous sulfate, exsiccate
Ferrous gluconate
Ferrous fumarate

20
30
12
33

Ferrous pyrophosphate
Ferrous carbonate

12
48

Common ADR: side effect GI

Parenteral Iron
products
Indications
Iron malabsorption
Intolerance of oral iron therapy
Noncompliance

Iron dextran (IM or IV)

Test dose of 25mg (IM or IV) over 510 min in
diluted IV solution
Monitor for 1 hour for adverse reactions
Allergic reaction including anaphylaxis

Goals
HgB rising 12 g/dL per week and continue
treatment at least 36 months

Vit B12 Def & Folate Def anemia

Inacti
ve

Clinical manifestations
Cardiorespiratory symptoms
Neuropsychiatric abnormalities (Delay & Only
B12)
Paresthesia
Ataxia
- Visual disturbances
Pernicious anemia

Goals
Signs and symptoms usually subside after
replace
Vit B12 or folate

Def.
Etiology
Treatment
Vit B12 - intrinsic factor - IM

Cyanocobalamin or
hydroxycobalamin

Folate

-
-
enterohepatic
circulation

Phenytoin, oral
contraceptive,
isoniazid

- Oral Vit B12 is

rarely indicated
-Oral folic acid 5 mg
OD

Hemolytic Anemia
G6PD (Glucose6Phosphate Dehydrogenase)
Deficiency
Genetic abn. X-link recessive
(sex??)
lack of glucose6phosphate
dehydrogenase

Symptoms of hemolytic anemia

Treatment
-

oxidative
stressors
Folic acid and iron
(Rarely)

Drug induced Hemolytic anemia

Antimalarials: Primaquine, Chloroquine,
Hydroxychloroquin
Antibiotics: Dapsone, Sulfasalasine,
Quinolone
Analgesics: ASA
Miscellaneous: Thiazide Diuretics, Vitamin
K, Penicillamine, Ascorbic (large dose),
Fava beans

Thalassemia
Definition
- *

- ,
-
(hemolytic anemia)

Pathophysiology

Fetal
Adult
Minor

HbF (22)
HbA (22) 98%
HbA2 (22) 2%

-Thalassemia

> + > E > 0

Type

Genotype

Phenotype

Minor

(0/) or (+/)

Hb > 10
Asymptomatic
carrier

Major

(0/0), (0/+) or (+/+)

Hb < 7

Intermediate

(0/+) or (+/+)

Hb 7-10

-Thalassemia
Type
silent carrier
minor

Genotype

Phenotype

(- /)

Asymptomatic

(- -/) , (- /-)

Asymptomatic

HbH disease

(- - / - )

Mild to mod. Microcytic

hypochromic

Hb Barts
hydrops

(- - / - -)

*(- -) thal 1, (- ) thal 2

Classification of Thalassemia
stem cell transplantation

Severe
Thalassemia

Homo. b
thal
b thal/Hb
E

high transfusion + iron

chelation
low transfusion + iron
chelation
splenectomy

Moderately
high transfusion iron
b thal/Hb E chelation
severe
low transfusion
Thalassemia
splenectomy

Hb H
low transfusion (hemolysis)
disease
b thal/Hb
*
E Gold: keep Hb > 10 g /dl
Mild
Thalassemia

Clinical manifestations

Iron chelation

ncrease RBC destruction

Pituitary gland Impaired

growth
Heart
cardiac failure
Liver
hepatic cirrhosis
Pancreas diabetes mellitus
Gonadal
infertility
Thalassemia & Iron overload


(T2*)
T2* < 20 ms LVEF

Indication

1. > 10-20
2. Serum ferritin > 1,000 ng/ml
Goal: NTBI

Treatment:
-Deferoxamine (DFO)
-Deferiprone (DFP)
-Deferasirox (DFS)

Agent

DFO

Ad
-Cont.

Half life

Clearance

20 min

urinary,

Infusion 50-

fecal

60

S/E
-Retinopathy
-Ototoxicity
-Growth

mg/kg/day

retardation

Add.Vit C

-Local skin
reaction
-Skin ulceration
-Yersinia
infection

-Oral, tid

3-4

-Neutropenia

Monitor
Audiometry and eye annually

DFO DFP DFS

/

CBC weekly

ALT monthly (first 3-6 mon), thenevery 6

months
ALT monthly

Serum creatinine monthly

Serum ferritin level quarterly

liver iron annually

 Reticulocyte index (%):

Total iron binding capacity (TIBC):

transferrin
Transferrin :
Transferrin saturation (TSAT):
transferrin
Serum ferritin: