17 year old with a known haematology disorder presents unwell.
She hasHaemolytic anaemia – low Hb, low PCV, high LDH, high conjugated bilirubin
Normal bone marrow function – high reticulocyte, high WCC, high platelets
Normocytic, normochromic anaemia
Normal renal function
Caused by significant underlying febrile disorder – viral/ bacterial etc
DDx of haematologic disorder (in this patient):
Hereditary haemolytic anaemia
- extravascular (in spleen) –
- H spherocytosis
- Sickle cell anaemia
- auto immune
- intravascular –
-
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
- Intramedullary –
- Pernicious anaemia
- Thalassaemia Major
Other (unlikely in this patient):
Acquired causes:
-
Toxic chemicals and drugs
-
Antiviral agents (eg, ribavirin)
-
Physical damage
-
Infections
- Immune disorders
intravascular (hereditary or acquired) –
- Prosthetic cardiac valves
-
Thrombotic thrombocytopenic purpura
-
Disseminated intravascular coagulation
-
Transfusion of ABO incompatible blood
-
Paroxysmal nocturnal hemoglobinuria (PNH)
- Haemolytic Uraemic Syndrome (HUS)
Complications include:
- due to anaemia,
- the extent of compensation,
- previous treatment,
- the underlying disorder.
Presenting Complaints/Complications:
- In intravascular hemolysis, iron deficiency due to chronic hemoglobinuria can exacerbate anemia and weakness
-
Tachycardia, dyspnea, angina, and weakness occur in patients with severe anemia, as cardiac function is sensitive to anoxia
-
Persistent hemolysis may result in the development of bilirubin gallstones; these patients may present with abdominal pain
-
Bronze skin color and diabetes occur in haemosiderosis;
-
iron overload may occur in patients who have received multiple transfusions or those who have been administered iron therapy erroneously
-
Dark urine may be due to haemoglobinuria
-
In addition to hemolysis, patients with thrombotic thrombocytopenic purpura (TTP) may experience fever, neurologic signs, renal failure, and thrombocytopenia
-
Leg ulcers may develop in patients with sickle cell anemia and other hemolytic disorders, as a result of decreased red blood cell (RBC) deformability and endothelial changes