Signs of anemia - Aplastic anemia
Sign of anemia - Aplastic anemia.
Aplastic anemia is associated with bone marrow aplasia. Aplastic anemia is known as deficiency of all blood cell element. It is also known as pancytopenia.
The etiology include idiopathic causes in 40% of people with aplastic anemia. It is associated with T cell immunological suppression of myeloid stem cell. The acquire causes include exposure to radiation, any chemical substance such as DDT or benzene, or any drugs such as alkylating agent, chloramphenicol, methotrexate and GOLD. It also includes viral infection such as HIV, CMV, measles, parvovirus and B19 and also a disorder known as paroxysmal nocturnal hemoglobinuria.
Aplastic anemia may also be inherited in case of dyskeratosis congenita ( skin and nail atrophy / sex linked disorder or Fanconi anemia. Fanconi anemia is a rare autosomal recessive disorder which leads to an impairment of DNA repair. Fanconi anemia is presented with familial aplastic anemia, microcephaly, bone abnormalities, renal abnormalities and hyperpigmentation of the skin as well as hypogonadism.
In term of epidemiology , it present at any ages and sexes especially male. The annual incidence of aplastic anemia are 2- 5 per 1 000 000.
The onset of the symptoms may months ( slow ) or fast/ rapid ( days ). The patient may present with the symptoms of anemia ( reduce in red blood cell count ) such as dyspnoea, lethargy, fatigue and leukopenia ( reduce in white blood cell count ) with an increase in severity and frequency of infection . The patient may also suffer from bleeding problems such as easy and recurrent case of bleeding, epistaxis or nose bleed and bleeding gum and bruising as a result of reduction in platelet count.
While examining the patient, the patient may appear pale ( sign of anemia) . present with bruises and petechia ( sign of platelet deficiency ) and develop bacterial and fungal infection ( sign of white cell deficiency)
Pathologically , the bone marrow appear to be white and pale macroscopically. Microscopically , the bone marrow may appear to be hypocellular. It consists of empty bone marrow space with fat cell and fibrious stroma and foci of plasma cell and lymphocytes . The arrangement is known as chicken wire appearance.
The investigation includes full blood count, blood film, bone marrow trephine biopsy, chromosomal analysis and Ham’s test.
Full blood count may reveals reduce level in hemoglobin, platelet and white cell count with normal MCV and low or absent reticulocytes. The blood film may exclude myeloma and leukemia. The bone marrow trephine biopsy may be performed to exclude other causes such as leukemia, lymphoma, myeloma, malignancies and myelofibrosis as well as any evidence of bone marrow infiltration. The chromosomal analysis my detect the present of Fanconi anemia that is characterizes by an increase in the random breaks of the peripheral lymphocytes. The Ham’s test is performed to measures the sensitivity of the red blood cell to lysis by complement following activation in acidify serum. It is useful to detect the paroxysmal nocturnal hemoglobinuria.
The management of aplastic anemia include treatment of the underlying causes which includes reviewing any drug intake that may precipitate aplastic anemia. The supportive therapy may include blood and platelet transfusion, antibiotic prophylaxis and antibiotic for infection. The immunosuppressive therapy may include ciclosporin A steroid , antithymocyte globulin and androgen ( oxymethalone ) for patient > 45 years old. Patient who is < 20 years old may required bone marrow transplant ( allogenic ) from HLA - matched sibling. Immunosuppressive therapy followed by bone marrow transplant may be required for patient between the age 20 - 45 years old.
The complication may include graft versus host disease and infection in bone marrow transplant patient as well as bleeding. Acute myeloid leukemia may develop in 10% of patient with Fanconi anemia.
The prognosis depends on the patient age and how severe the pancytopenia is. Poor prognostic indicator includes:
reticulocytes < 40 * 109 /L
neutrophil < 0.5 * 10 to the power of 9 /L
platelet < 20* 10 to the power of 9/L
If these features lasting more than 3 weeks mortality is 50%. Transplantation of bone marrow appear to be curative.
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