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Aplastic anaemia

Updated on June 13, 2011

Aplastic anaemia

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

predisposition

♥Constitutional aplastic anaemia

4Fanconi type

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

chemicals

viral and bacterial infections

In about half of cases, no cause can be found, immune defect of T cell is suspected

in these.

Clinical features

-Anaemia

-Bleeding manifestations

-Recurrent infections

Investigations

-FBC -Anaemia

-Leucopenia

-Thrombocytopenia

-Serum iron & TIBC

-Bone marrow biopsy (hypocelluler)

Treatment

-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.

Blood requirement

(12-pretransfusional Hb) X body weight X 4 ÷ 2

= (12-6) X 16 X 4 ÷ 2

= 6 X 64 ÷ 2

= 384 ÷ 2

= 192ml

Maximum blood transfusion is

= 20ml/kg/day

= 20 X 16

= 320

192ml for two days


Why chelation?

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,

Cardiac failure

●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),

Skin colour

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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