Aplastic anaemia

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


Comments

No comments yet.

    Sign in or sign up and post using a HubPages Network account.

    0 of 8192 characters used
    Post Comment

    No HTML is allowed in comments, but URLs will be hyperlinked. Comments are not for promoting your articles or other sites.


    More by this Author


    Click to Rate This Article
    working