地中海貧血 Thalassemia


地中海貧血Thalassemia

Thalassemia, in simple terms, that is the insufficient amount of blood in the body.Due to the blood is “the transportation" to transport oxygen and nutrients ,If “the frequency" does not adequately, the body naturally does not have sufficient nutrients.If only a little, not on the health effects, but if severe, may be a danger to health.

Thalassemia is one of the hereditary anaemia. As the patient has a problem of their own manufactures hemoglobin to lead to inability to produce red blood cells, resulting in chronic hemolysis.That is formation of anaemia.

Thalassemia is mainly popular in Eastern Mediterranean and other tropical and subtropical regions such as the Middle East and Southeast Asia and other countries.

Pathology of Thalassemia

1) Red blood cell’s main function is to transport oxygen around the body to all organizations. Hemoglobin is an important element in red blood cells.

2) Bone marrow is the organs of making the red blood cells.Under the stimulus of the anemia in the long run,the thalassemia patient’s bone marrow is particularly active but it cannot effectively produce and red blood cells.

3) Abnormal hemoglobin in red blood cells will accelerated decomposition in the human body(hemolysis).

4) Due to the red blood cells and the hemoglobin are not enough, the tissue of whole body is not working with low oxygan.

 

In the case of pathological, there are several types of thalassemia. Depending on the clinical severity of the disease, it can be classified into thalassemia major, thalassemia intermedia and thalassemia minor. The Alpha-thalassemia and Beta-thalassemia is common in Hong Kong.

● Thalassemia major

Thalassemia major is lack of normal hemoglobin to lead to severe anaemia.Patient is rely on blood transfusions to keep alive.Without the blood added that patients will not only look pale marking, fatigue, loss of appetite and other symptoms of anemia, more vulnerable and slow-growing, leading to Dysplasia, skeletal deformation and heart failure, etc, so that patient’s life may be less than ten years old.

● Thalassemia intermedia

Thalassemia’s condition between major and minjor, thesymptoms of anemia is serious than minor, but there is no obvious symptoms, body development as usual, have no regular blood transfusions. However, in some cases, for example bacterial infection, oral drugs or pregnancy, etc, anemia can exacerbate, and need to supplement the blood.

● Thalassemia minjor

Such patient have poor thalassemia genes but only the blood manufacture function have minor effects.Anemia will be very light, or even no,the health is not affected.

【Genesis】

It is a hereditary disease, by two names with the same type of thalassaemia gene couples open their next generation

● The Alpha-thalassemia

About 5% of people with alpha thalassaemia genes, when most were thalassemia minor, in patients with small parts to be thalassemia intermedia. Due to the alpha thalassaemia patients were fall ill when they are prenatal. Most are stillborn or quickly die after birth, so only a few alpha thalassemia major patients in worldwide are able to survive and grow up.

● The Beta-thalassemia

About 4% of Hong Kong people with the beta-thalassemia factor. As alpha-thalassemia patiants are mostly thalaessmia minor, small parts to be thalassemia intermedia. At present, Hong Kong is about 400 to 500 of patients are bate thalassemia patiant.

The Beta-thalassemia major patiants in the newborn within a few months of normal health, haven’t any symptoms. Typically the severe anaemia will seen when the 3-8 months after , and begin to need to blood transfusions.

【Symptoms】

Basically, it will know the baby whether suffering from the thalassemia major shortly after birth.

【Therapy】

● Thalassemia major

1) Regular blood transfusions

2) Chronic drug injections of iron removal – Because of red blood cells rich in iron and long-term blood transfusions will cause a lot of accumulation of iron in the body, it will

3) Drugs stimulate the growth of red blood cells.

4) Transplantation therapy – Pulled out of the bone marrow or blood stem cells from the patient’s brothers and sisters and transplant to patients. Patients with bone marrow hematopoietic function will returns to normal, in recent years there have been many successful examples in Hong Kong.But because the donor must not suffer from severe anemia and anastomosis with patient organizations, on average, only one third of patients with severe anemia can have access to transplantation.

 

● Thalassemia intermedia

1) Supply the Vitamins – Because their bone marrow is active than ordinary people, so they need to supplementary vitamins needed to cope with.

2) May need to have a blood transfusion

3) Still need to use the drugs to avoid daily absorb large amounts of iron affect the health of the body.

Thalassemia major and Thalassemia intermedia patients need to pay attention ﹕

1) Do not eat drugs carelessly, otherwise it may aggravated anemia.

2) Maintain a balanced diet.

3) Maintain regular exercise.

4) Should visit the doctor when you feel sick.

【Preventive】

Thalassemia is a genetic disease, prevention must be at the beginning of children prenatal.
When two of the same type thalassaemia patient combination , had a chance to give birth to patients with severe anemia, therefore, you should accept the blood tests before pregnancy. we will know whether the baby is it suffer from Thalassemia.

● If the parents belong to the Beta-thalassemia minjor

–Fetus has one fourth opportunity to get fully functional.

–One second opportunity to become thalassemia minjor.

–One fourth opportunity to become thalassemia intermedia or major

● If the parents belong to the Alpha-thalassemia

The Alpha-thalassemia genetic complexity, parents must take a detailed genetic analysis to forecast will the baby become thalassemia intermedia or major.

In this case, can still be pregnant but the fetus must be receiving prenatal check to verify whether the fetus in patients with severe anemia.
Due to laboratory tests take times, the antenatal checks must doing in early pregnancy. When pregnancy beyond 24 weeks, the fetus is considered an independent life, even that is verify the baby is suffer from thalassemia, we can not take abortions also.

至於重型甲型地貧的胎兒,他們絕大部分出生後也不能生存,故即使到懷孕後期,在父母的意願下仍可人工終止懷孕。

雖然如此,終止懷孕的手術亦應儘量在懷孕早期進行,以避免不正常胎兒對孕婦產生不良的影響。

 

資料來源:(明報)http://www.mingpaohealth.com/cfm/archive3.cfm?File=Archive/diseases/blood02.txt


地中海貧血Thalassemia

貧血,簡單而言,即是身體內的血液量不足,由於血液是運送氧氣和養份的「交通工具」,在「班次」不足,身體自然沒有充足的養份,若只差少許,對健康的影響還不大,但若情況嚴重,則可能危及健康。

地中海貧血病是其中一種遺傳性的貧血病。由於患者自身製造血紅蛋白的功能出現問題,以致無法生產紅血球,結果有長期溶血的現象,形成貧血。

這病主要流行於地中海及其他熱帶和亞熱帶地區,例如中東和東南亞等國家,故命名為地中海貧血症。

地中海貧血的病理

1) 紅血球的主要功能是把氧份運送到全身所有組織,而血紅蛋白是紅血球內的重要原素。

2) 骨髓乃製造紅血球的器官,在長期貧血的刺激下,地中海貧血病人的骨髓特別活躍,但卻不能有效地生產及放出紅血球。

3) 含有不正常血紅蛋白的紅血球人體內加速分解(溶血)。

4) 沒有足夠的紅血球和血紅蛋白,全身的組織便因沒有足夠的氧份而不能正常操作,構成貧血現象。

 

在病理學而言,涉及血紅蛋白的病變有很多種,故地中海貧血症亦可分為多種類型,當中以甲型和乙型較常見於本港。而根據臨床病情的輕重程度,地貧又可分為重型、中型及輕型。

● 重型地中海貧血

患者身體極缺乏正常的血紅蛋白,導致有嚴重的貧血,終身也須依賴定期輸血活命。若沒血液補充,患者不單會有臉色蒼白﹑容易疲倦﹑食慾不振等貧血病徵,更會易受感染和生長緩慢,導致發育不全﹑骨骼變形和心臟衰竭等,這樣患者的壽命可能不到十歲。

● 中型地中海貧血

病情介乎重型和輕型之間,貧血病狀較輕型嚴重,但也沒有很明顯的病徵,身體會如常發育,亦無需定期輸血。但在某些情況下,例如感染細菌﹑誤服藥物或懷孕等,貧血情況可能會加劇,而需要補充血液。

● 輕型地中海貧血

這類患者雖然有「地貧」的遺傳基因,但製造血紅的功能只受輕微的影響,故貧血現象亦會很輕,甚或沒有,健康基本上不受影響。

【成因】

遺傳性,由兩名帶有同一型的地貧基因的夫婦傳結他們下一代。

● 甲型地中海貧血

約有5%的港人帶有甲型地貧的遺傳基因,當大多數是輕型,小部份則屬中型患者。由於甲型重性地貧患者在胎兒期已病發,故大部份會胎死腹中或出生後迅速夭折,因此全球只有數名甲型重型地中海貧血病人能生存和長大。

● 乙型地中海貧血

本港大概有4%的市民帶有乙型地中海貧血病的因子。如甲型病者一樣,當中大部份屬輕型,小部份屬中型。現時全港大概有400至500名乙型重型貧血患者。

乙型重型貧血病者在初生的數月內健康正常,沒有病徵。通常到了三至八個月大時才會嚴重貧血,並開始需要輸血。

【病徵】

基本上出生不久便會知道嬰兒是否患上重性地中海貧血。

【治療】

● 重型地中海貧血

1)定期輸血

2)長期注射除鐵藥物

紅血球含有豐富的鐵質,長期輸血會令大量鐵質在體內積聚,對多組器官造成破壞,故必須注射除鐵藥,防止鐵質積聚,否則病人可能活不過二十歲。

3)用藥物刺激紅血球生長。

4)移植療法 – 從同父同母的兄弟姊妹抽取骨髓﹑臍帶血或血液中的幹細胞,移植到患者的身上,令患者的骨髓回復正常的造血功能,得以痊癒,近年本港已有不少成功的例子。

但由於捐贈者必須沒有患重型貧血及與病人組織吻合,平均來說,只有三分一的重型貧血病人能有機會接受移植治療。

 

●中型地中海貧血

1)補充維他命

由於骨髓會較平常人活躍,故需要補充維他命應付所需。

2)間中可能要接受輸血。

3)仍要使用除鐵藥物,以免身體日常吸收大量鐵質影響健康。

兩種地貧患者另外需注意﹕

1)不要亂吃藥物,否則可能會令病情加劇。

2)保持均衡飲食。

3)保持適量運動。

4)病向淺中醫,遇不適應立即看醫生。

【預防】

此病屬遺傳性質,預防必須在孩子未出生前開始進行。

只有兩名屬同一類型的地貧患者結合,才有機會生下重型貧血患者,因此,只要在懷孕前接受血液化驗,便知道胎兒是否有機會患上地中海貧血。

● 若夫婦倆同屬乙型輕型貧血患者

–胎兒有四分一的機會完全正常

–二分一的機會成為輕型地貧患者

–四分一的機會成為中型或重型貧血患者

● 夫婦帶甲型地貧基因

甲型地貧的遺傳較為複雜,夫婦必須接受詳細的基因分析,才能預測下一代成為中型或重型地中海貧血患者的機會。

若出現上述情況,夫婦仍可懷孕,但胎兒必須接受產前檢查,以證實胎兒是否重型貧血患者。

由於化驗結果需時,產前檢查必須於懷孕初期進行,否則當懷孕到了24周以後,胎兒已被認為是一個獨立生命,即使胎兒證實是乙型重型地中海患者亦不能進行墮胎手術。

至於重型甲型地貧的胎兒,他們絕大部分出生後也不能生存,故即使到懷孕後期,在父母的意願下仍可人工終止懷孕。

雖然如此,終止懷孕的手術亦應儘量在懷孕早期進行,以避免不正常胎兒對孕婦產生不良的影響。

 

資料來源:(明報)http://www.mingpaohealth.com/cfm/archive3.cfm?File=Archive/diseases/blood02.txt

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