Aplastic anaemia is characterized by a reduction or absence of haemopoitic elements in all cell lines in the bone marrow leading to peripheral blood pancytopenia.
Usually in adult - acquired
In children - idiopathic or one of several constitutional forms with an inherited
♥Constitutional aplastic anaemia
Aplastic anaemia is present with other stigmata.
Eg: Brownish pigmentation of the skin, hypogonadism, microcephaly, short
stature, skeletal defects on the radial side.
Face - micropthalmia, depressed and wide nose bridge
♥Acquired aplastic anaemia
Certain agents given in a sufficient dose depress the bone marrow of all individuals.
Eg: antimetabolics, chloramphenicol, NSAID, antiepileptics
viral and bacterial infections
In about half of cases, no cause can be found, immune defect of T cell is suspected
-Serum iron & TIBC
-Bone marrow biopsy (hypocelluler)
-Maintain Hb over 7g/dl
-Treat the infections
-Bone marrow transplantation is the treatment of choice
but it need HLA matched compatible sibling donor &also it cause cost effect
-Immunotherapy with antithymocyte globulin (ATG), antilymphocyte globulins
(ALG) & high doses of dexamethasone also show promising results with
improvements occurring in around 2/3 of cases.
Prognosis is poor
Sepsis & haemorrhage are the main causes of death.
(12-pretransfusional Hb) X body weight X 4 ÷ 2
= (12-6) X 16 X 4 ÷ 2
= 6 X 64 ÷ 2
= 384 ÷ 2
Maximum blood transfusion is
= 20 X 16
192ml for two days
Iron overload cause many problems in many organs.
So Desferioxamine have to be start when ferritin level is about 1000mg/l
(at 10th transfusion)
At hospital who are not having infusion pump IV Desferioxamine dose =100mg /kg /day for 3days. Vitamin C is given to chelation.
At home by infusion pump
40mg/kg/day for 6days per week.
Iron overload can lead to multiorgan dysfunction.
● Endocrine failure
Short stature, delayed puberty, oestrogen deficiency, hypothyroidism,
Diabetes mellitus & hypoparathyroidism
●cardiac involvement – cardio myopathy, arrhythmias,
●Hepatic involvement – cirrhosis, fibrosis
So to identify above conditions routine investigations have to be done as follows
Monthly – weight, height, urine sugar
Once in 3 months – USS (to assess the status of liver & spleen),
Once in 6 months – serum iron, TIBC, serum ferritin
Annually - Echocardiogram
Eye & ENT referral
Hormone levels – GH, T3, T4, TSH, PT
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