Aplastic anemia - Acute
Definition
A rare hematopoietic disorder, most often due to pluripotent stem cell injury, that results in pancytopenia and hypocellular bone marrow
Findings
* High output heart failure
* Systolic flow murmur
* Angina
* Dyspnea on exertion
* Bleeding gums
* Easy bruising
* Epistaxis
* Excessive and frequent menstruation
* Fatigue
* Fever
* Headache
* Petechiae
Tests
* Peripheral blood smear examination, light microscopy: Blood smear examination is indicated in the initial evaluation of suspected or unexplained anemia.
* Bone marrow biopsy, needle or trocar: Bone marrow biopsies allow for analysis of bone marrow contents and cellularity to aid in diagnosis of bone marrow disorders.
Complete blood counts show pancytopenia with preservation of lymphocyte count in aplastic anemia. In early cases isolated thrombocytopenia may be the only finding on CBC. The anemia is often macrocytic with reticulocytopenia.
* Complete blood count with white cell differential, manual
Severe pancytopenia may be seen with severe folate deficiency. If a folate deficiency megaloblastic anemia is discovered, it must be treated prior to making a diagnosis of aplastic anemia .
* Folic acid measurement, serum
Suspected or known anemia
* Reticulocyte count: The reticulocyte count is essential for differentiating between anemias arising from nonfunctional versus functional bone marrow .
Suspected vitamin B12 (cobalamin) deficiency secondary to pernicous anemia
* Serum vitamin B12 measurement: Serum cobalamin levels below 74 pmol/L indicate a probable cobalamin deficiency .
Chest x-rays are useful in diagnosing patients with aplastic anemia who present initially with infection.
* Plain chest X-ray
Post-hepatitic aplastic anemia can occur 2 to 3 months after an acute episode of hepatitis. Liver function testing, along with hepatitis A antibody, hepatitis B surface antigen, and hepatitis C antibody, can aid in diagnosis.
* Hepatic function panel
All patients with aplastic anemia should be screened for systemic lupus erythematosus (SLE) with antinuclear antibody (ANA) and double-stranded DNA antibody (dsDNA). Pancytopenia may occur with SLE if the autoimmune process involves bone marrow or in the rare occurrence of SLE-induced myelofibrosis.
* ANA measurement
All patients with aplastic anemia should be screened for systemic lupus erythematosus (SLE) with antinuclear antibody (ANA) and double-stranded DNA antibody (dsDNA) testing. Pancytopenia may occur with SLE if the autoimmune process involves bone marrow or in the rare occurrence of SLE-induced myelofibrosis.
* Antibody to double stranded DNA measurement
Patients with Fanconi anemia have abnormal or displaced kidneys on abdominal ultrasound. Also, malignant hematologic disorders that cause pancytopenia may cause splenomegaly and/or enlarged lymph nodes, which may be found via ultrasonography.
* Abdominal ultrasound
Differential Diagnosis
* Myelodysplastic syndrome - Chronic
* Paroxysmal nocturnal hemoglobinuria
* Common acute lymphoblastic leukemia
* Hairy cell leukemia
* Anorexia nervosa - Chronic
* Mycobacteriosis
* Malignant lymphoma
* Myelofibrosis
* Acquired aplastic anemia
* Fanconi's anemia
* Amegakaryocytic thrombocytopenia
* Shwachman syndrome
* Dyskeratosis congenita
Treatment
Drug Therapy
Aplastic anemia
ANTITHYMOCYTE GLOBULIN EQUINE
Adults: Antithymocyte globulin 15 mg/kg IV over 12-18 hours for 5 days (test dose of 1 mg/100 mL solution must be given prior to initiating 5-day therapy); and on fifth day ADD prednisolone 1 mg/kg/day IV for 9 days, then tapered for 5 days; and on day 14 ADD cyclosporine 5 mg/kg/day orally until blood counts plateau (trough levels maintained between 150 and 250 mcg/L); after 6 months at plateau a very slow tapering can be initiated; at least 3 months required for a response, a second course of therapy may be repeated at this time if first course was unsuccessful
CYCLOPHOSPHAMIDE
Adults: 45 mg/kg IV for 4 days
FILGRASTIM
Adults: 5 mcg/kg/day subcutaneously
Aplastic anemic patients at risk for Aspergillus infections
FLUCONAZOLE
ITRACONAZOLE
Procedural Therapy
* Bone marrow transplant: Bone marrow transplantation is used to treat aplastic anemia, severe myelodysplastic disorders, and infiltrative malignancies of the bone marrow .
* Transfusion of packed red blood cells: Patients with a HCT >30% rarely require RBC transfusion; patients with acute anemia and a HCT <21% typically require transfusion .
Non-Procedural Therapy
Aplastic anemia
* Self Care
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