86. 足月產新生兒第一天血液檢查結果如下: RBC 4.95x106/mm3, Hb 14.6g/dl, MCV 88fl, RDW 14.8%, HbA 8.5%, HbF 85%, Hb Bart’s 6.5%, 最可能的診斷為？
(A)正常範圍。
(B)alpha型輕型海洋性貧血 (alpha-thalassemia minor)。
(C)beta型輕型海洋性貧血 (beta-thalassemia minor)。
(D)alpha型中型海洋性貧血 (alpha-thalassemia intermedia)。
(E)alpha型重型海洋性貧血 (Hb Bart’s hydrops fetalis)。

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統計： A(6), B(23), C(2), D(10), E(4) #1125793

詳解 (共 1 筆)

#1380543
Alpha thalassemias:
Carrier              : −,α/α,α               ;  Newborn: 1-2% Bart(gamma_4) , adult: normal   ;   no anemia
Trait(minor)       :  −,α/−,α −, −/α,α ;  Newborn: 5-10% Bart(gamma_4) , adult: normal  ;  mild anemia
HbH(intermedia):  −,−/−,α              ;  Newborn: Bart: 20-30% , Adult: HbH(beta_4) ; mild anemia, transfusions not required
Constant srping : −,−/α,α^CS         ; 2-3% constant spring, 10-15% HbH(beta_4)  ; moderate to severe anemia, transfusion, splenectomy
Major : -,-/-,-  ;  Hb Barts(beta_4), Hb Portland(delta_2 Beta_2 or delta_2 gamma_2), HbH(beta_4) ;
                        abscent HbA, HbF and HbA2 ; 
                        hydrops fetalis
Beta thalassemias:
Trait/Silent:  beta+/A or beta0/A or beta+/beta+ ; elevated HbA2, variable elevation of HbF ; microcytosis, no or mild anemia
Intermedia: beta+/beta+ , others*  ; elevated F and A2, trace A ; usually dose note require transfusion in childhood
Major: beta+/beta+, beta0/beta0, beta0/beta+, E/beta0 ; elevated F and A2 ; transfusion dependent
Dominant(rare): B0/A , elevated F and A2, transfusion dependent
 
Derived from Nelson 20ed Table 426-7
*: may also beta+/beta0, beta+/E (Uptodate)

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