ETIOLOGY S&S Ix Tx

HEMATOLOGY
1. RBC
o ANEMIA
- Hb : < 10 g/dl (≥15g/dl)
- Hct : < 30% (≥45%)
- RBC : <2.8 M/mm3 (≥5 M/mm3) (MCV, MCH, MCHC, RDW)

 Low MCV (<80) : MYCROCYTIC HYPOCHROMIC “TAILS”

1) Thalassemia See in Hemolytic Anemia
2) Anemia of Chronic Infections Symptoms of anemia Iron indicies U/D
disease Malignancy - - ↓ Serum Fe Erythropoietin : in CKD
Inflammatory - ↓ TIBC
Rheumatologic : RA, SLE - ↑ Ferritin
Chronic renal & liver Peripheral blood
disease Bone marrow
Endocrine dysorders : DM, - N/↑ Fe store
hypothyroidism - ↓/- Fe in erythroid
precursors

3) Iron Deficiency >>demand : pregnancy Symptoms of anemia Iron indicies - Oral

Anemia <<iron : GI bleeding, - Fatigue - ↓ Serum Fe (<50 mg/dl) Ferrous sulphate 200-325 mg 3x1(Fe 65
chronic menorrhagia - Headache - ↑ TIBC >350 mg/dl mg)
- Light-headedness - ↓ Ferritin <20 µg/L Feerous gluconate 300 mg 3x1(Fe 35 mg)
- Dizziness - ↓ Trasnferrin sat. <15% Ferrous fumarate 300 mg 3x1(Fe 100 mg)
- Syncope Peripheral blood smear + Vitamin C เพิม ่ การดูดซึม
- Dyspnea - Hypochromic microcytic - IV (iron sucrose/Dextran)
- Palpitations - Pencil forms - Transfussion (Hb<6)
- Anisocytosis *3 Mo. Until Ferritin normal (≥100)
Pica - Target cells *evaluate : Reticulocye, Hb (>10)
Angular stomatitis Bone marrow (gold *↑: เนื้อ, fructose (น้าผึง้ เบอรรี)่ , citric acid
Atrophic glossitis standard but rarely done) (ส้ม มะนาว) วิตามินซี
Koilonychia (spoon nail) - Iron stain (Prussian blue) *↓: antacide, phytate (ถั่วเมล็ดแห้ง งา),
Dysphagia : ↓ Fe in macrophages & tannin (ชา องุน ่ กล้วย)
in erythroid precursors
4) Lead Poisoning Lead Abdominal pain Blood lead > 80 µg/dL Chelation therapy (ล้างพิษหลอดเลือด)
Constipation LEAD 1st line :Dimercarpol & EDTA
Irritability
Difficulty concentrating
 - Lead lines on gingivae &
epiphyses of long bone
on x-ray
- Encephalopathy &
Erythrocyte basophilic
stippling
- Abdominal colic&
microcytic Anemia
- Drops (wrist &foot drop)
5) Sideroblastic Defect in heme Symptoms of anemia Iron indicies - X- linked : high dose Pyridoxine
Anemia biosynthesis in erythroid Hepatosplenomegaly - ↑ Serum Fe2+ (Vit.B6)
percursors (iron over load) - N TIBC - Acquired : EPO & G-CSF
Genetic : - ↑ Ferritin - Reversible : remove precipitating cause
- x-linked - ↑ sTfR - Transfusions for severe anemia
- Autosomal Dominant Blood film/ bone marrow
Acquired : biopsy
- Myelodysplasia - Ringed sideroblast
syndrome (MDS) - Hypochromic
Reversible : - Anisocytosis,
- Drugs (INH, poikylocytosis,
chloramphenicol) basophilic stippling
- Alcohol
- Lead
- Zinc toxicity
- Copper def.
- Hypothyroidism
 Normal MCV (80-100) : NORMOCYTIC
Cause : ABCD
Acute blood loss
Bone marrow failure
Chronic disease
Destruction (hemolysis)
o Low Reticulocyte
(<2%)
1) Aplastic anemia
o High
Reticulocyte(>2%)
1) Hemolytic anemia
- Hemoglobinopathy
a) Sickle cell Sickle cell
anemia (S-Beta
Thalassemia)
b) Thalassemia Defect of hemoglobin 2α + 2β = HbA (N: >95%), 2α + 2δ = HbA2 (N 1.5-3.5%), 2α + 2γ = HbF
chain
Hereditary (Recessive 1) β-Thalassemia
Autosomal)  β-thal major (cooley anemia)
- Phenotype : - Present in 2nd Mo : Progressive anemia, hypersplenism, cardiac decompensation
 Major : transfussion - Expanded medullary space with increase expansion of face (facies cooley) and skull (hair on
depent end app.),
 Intermedia : mild - Secondary hemochromatosis : ABCDH (A: Athralgia, B : Bronze skin, C : Cardiomyopathy, D
symptom : Diabetes, H : Hyppogonadism)
 Minor/Trait : - Extramedullary hematopoiesis --> hepatosplenomegaly
asymptomatic - Severe --> jaundice, pigment gall stone
- Genotype : - Ix :
 β-Thalassemia :  Hb < 6
chromosome 11  Peripheral blood film : teardrop, target, mycrocytic hypochromic, basophilic stippling
(mutation)  Hb electrophoresis : ↓HbA1 0-10%, ↑HbA2 >2.5%, ↑↑↑HbF 90-100%
 α-Thalassemia : - Tx :
chromosome 16  Lifelong regular transfusions : suppress endogenous erythropoisis
(delesion)  Iron chelation (deferoxamine, deferasirox) : prevent iron overload
 Folic acid (if not transfused)
Ix :  Splenectomy
CBC :
 Bone marrow transplant
- ↓ Hb, MCV, MCH,
 β-thal Intermediat
MHCH, ↑ Rt, RDW
- Severe anemia
Peripheral blood smear
- No transfusion need
“THAL”
 β-thal minor
- T : Target cell
- Asymptomatic
- H : Howell-jelly body
- ↑ HbA2 : 3.5-5% , ↑HbF 5%
- A : Anisocytosis
- No treatment
- L : Low MCV
2) α-Thalassemia
(Mycrocytic
 1 gene : α-thal 2/ silent carrier state : asymptomatic
hypocrhomic)
 2 gene : α-thal 1/α-thal carrier state/α-thal trait
Hb electrophoresis
- Genotype cis 2(- -/ αα) : Asians
- HbF
- Genotype trans 2(- α/-α) : African Americans
- HbA2
 3 gene : HbH disease : Heiz bodies, sever anemia
Hyperbilirubinemia
 4 gene : Hydrops fetalis/ Hb Bart/α-thal major : lethal in utero
 - Membranopathy
a) Hereditary Osmotic fragility test
spherocyte Spherocytes
- Enzymopathy
a) Glycotic
enzyme
b) G6PD G6PD assay
Bite cell
Heinz bodies
- AIHA
 High MCV (>100) : MACROCYTIC
o Megaloblastic
1) B12 deficiency
2) Folate deficiency
o Non megaloblastic
1) Alcoholism
2) Liver Disease
3) Reticulocytosis
- Polycythemia Vera
- Porphyria
- Blood Transfusion
Reactions
2. WBC
- Neutrophilia
- Neutropenia
- Lymphocytosis
- Lymphopenia
- Eosinophila
3. Hemostasis disorders
- Primary
a. Platelet
 Thrombocytopenia
1) ITP
2) TTP/HUS
3) HIT
 Thrombocytosis
b. vWD
c. Vascular
 1) HSP
- Secondary
1) Hemophilia
2) Vitamin K
Deficiency
3) DIC
4. Malignancy
- Lymphoid disorders
a. Leukemia
1) ALL
2) CLL
b. Lymphoma
1) Hodgkin
2) Non-Hodgkin
c. Plasma cell dyscrasias
1) Multiple Myeloma OLD-CRAB CT : < 70 yrs : autologous bone marrow
- Old : > 60 - Cells infiltrate bone transplant
- C : Calcemia elevate marrow ≥ 70 yrs : Melphalan & Prednisone
> 2.8 mmol/L - Activate osteoclast
- R : Renal - Lytic lesions
involvement (Cr > - Weak bone
176 mmol/L) IgA, IgG : elevate
- A : Anemia monoclonal
- B : Bone lytic lesion/ Bone marrow biopsy >
Back pain/ Bone pain 10% monoclonal
2) MGUS
3) Waldenstorm’s
macroglobulinemia
- Myeloid disorders
a. Leukemia
1) AML
2) CML
b. MPNs
1) PV
2) ET
3) CML
4) IMF
c. MDS