ANAEMIA -APLASTIC ANAEMIA its classification,causes,clinical feature,diagnosis,differential diagnosis,and newest treatment

ANAEMIA

Definition :

Physiologic: reduction in 02 transport of the blood .

Practically : lowering of HB concentration of the blood below the normal range for population of comparable age and sex

classification

(1) Failure of erythropoiesis .

(2) Shortened survival of the RBCs

(3) Loss of blood either due to internal or external haemorrhage

APLASTIC ANAEMIA

Definition :

Pancytopenia caused by hypoplasia of the bone marrow .

It may be severe (aplastic) or moderate (hypolastic).

Divided into two groups :

1- Aplasia follows exposure to certain chemical and physical agents (secondary ).

2- Idiopathic or (primary ) : no cause .

The more important cause of marrow aplasia

1-  Metals .

2-  Antimicrobial agents .

3- Anti –inflammatory drugs .

4- Anticonvulsant .

5- Antithyroid drugs .

6- Hypoglycemic agents .

7- Diuretics .

8- Various drugs and chemical agents .

9- Physical agents .

10- All cytotoxic drugs

It may be reversible or irreversible .

Clinical feature

. Acute or chronic .

. Occure at any age .

. Onset is usually insidious .

. First symptoms are usually those of anaemia (breathlessness , plapitation , anginal pains )

.Bleeding because of thrombocytopenia : epistaxis , bleeding gums , uterine losses , haemoptysis , while meningeal or intracerebral haemorhage is acommon terminal event.

Haemorrhage in the optic fundi are very common .

Petechial haemorrhage , ecchymoses in the skin when present are usually most marked on the legs .

.In other patients the initial symptoms are due to

GRANNULOCYTOPENIA :

Respiratory infection , infective episodes , serious infection (septicemia , pneumonia and

osteomylitis )

.Ulcerative lesions about the throat and pharynx and similar lesions may occur about the vulva and the anus .

.Patients usually show an intense waxy pallor without any hints of jaundice.

Diagnosis :

Suspected in a patients typically presenting with symptoms be related to his anaemia , neutropenia and thrombocytopenia and there is no lymphadenopathy or splenomegaly .

A marrow trephine and smear confirms

Differential Diagnosis :

1- Subacute leukemia or preleukemia blast cells .

2- Paroxysmal nocturnal haemoglobinuria : positive ham’s tests .

3-Bone marrow infiltration by metastastic malignancy .

4- Pure red cell aplasia : exclude other cause of anaemia +thymic tumour in most cases .

Investigations:

* Peripheral blood :

Anaemia , granulocytopenia , and thrombocytopenia .

The anaemia is usually normochromic and normocytic .

. Anisocytosis , poikilocytosis .

. The reticulocytes count is often very low .

.Serum iron level is usually high , total iron binding capacity is depressed , shortening of the red cell  life –span .

The leucopenia

Mainly affects granulocytes sometimes lymphopenia as well .

The platelet count

is often extremely low .

** The bone Marrow :

       aplastic ,consists of a few lymphocytes and scanty islets of surviving myeloid tissue .

Treatment :

1- Regular blood counts must be done when patients are such high-risk drugs as cytotoxic agents .

2- The mainstay is blood transfusion .

3-Bone marrow transplantation for children and adult below 40 years , from a compatable donor .

4- steroid therapy is sometimes of value .

5- Androgenic steroids : remarkable remissions in children .

6- Meticulous nursing care

7- Proper antibiotic therapy

Prognosis :

Very variable clinical course , 50 % of patients survive more than 6 months after the diagnosis .

A permanent remission may be expected in about 25 % .