Page 337 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

CHAPTER 17 ■ Hemoglobinopathies and Thalassemias 321

. B m , m . A m m

m m k m - m

- m m .

, , .

I , - m x Hem atopoietic Stem Cell

SCD . H m m (HSC)-

H x m m m m m m -

q m q . , x m ,

H , m x m m- SCD, β m .

, m

m , m Prevention

H F . B - G m SCD.

, W m SA ( ), -

m k m m

m m . k z DNA m m .

Ex m H F
Novel Pharm aceutical Therapies - m m - . S -

C , x FDA- m x

SCD. S m m

m SCD m . M m - k - -

. N : m m

m . R
■ m SCD ,

■ m H S m z .

■ x

■ - mm .

N x ; - mm SICKLE CELL SYNDROMES:
, x m , ; - PA HOGENESIS AND NEW APPROACHES

. B m x SCD m z . T
SCD, k SCD q H S

q SCD. I m k . T -
m m m m H S .

m m .
.

Bone Marrow Transplant

B m m SCD Sickle b Thalassemia

E . C T k m β
m m ( . ., m m m

k , m , ) z : k β m (F . . ). T -
HLA-m - m

. A x m % m k M . P

TABLE 17.2 Various Clinical States Associated w ith the Presence of Hb S

Clinical Severity

Genotype Percentage Hb S Percentage Non-S Hb (Scale 0–4) Other Clinical Features

SS 80–98 2–15 (fetal) 3–4 10%–20% reticulocytosis; rare

splenomegaly

S β thalassemia 60–90 10–30 1–3 Splenomegaly; microcytosis;

4+ target cells

SC 50 50 (hgb C) 1–3 Splenomegaly, 4+ target cells

AS sickle trait 30–40 60–70 (hgb A) 0 Normal morphology; no

splenomegaly