and Paul Telfer2
(1)
Department of Haematology, Guy’s and St Thomas’ Hospital, London, UK
(2)
Department of Haematology, Royal London Hospital, London, UK
Introduction
Chapter 2 covers normal ‘steady state’ levels for hemoglobin and their variation with age. The majority of patients with HbSS have steady state hemoglobin levels between 60 and 90 g/l. This anemia is a feature of SCD and is usually well tolerated without the need for routine transfusion. The majority of specialists would not recommend routine treatment for anemia even if the steady state level is <60 g/l but transfusion in these cases may be needed if other chronic complications develop, or if there is an acute drop in hemoglobin during a crisis. Older patients, especially those with pulmonary or cardiac disease, may not tolerate low hemoglobin levels and usually benefit from regular simple transfusion.
Acute Anemia
Clinical Presentation
Increasing anemia may lead to symptoms of tiredness, fatigue and headache. With increasing severity, there is pallor, exercise intolerance, shortness of breath, ankle swelling and eventually circulatory collapse. A slowly progressive anemia is better tolerated than anemia of rapid onset.
Causes and Investigation
The major causes of acute anemia are outlined in Table 14.1, laboratory investigations for determining the cause of anemia in Table 4.3 and a diagnostic algorithm in Fig. 14.1.
Table 14.1
Causes of acute anemia
Cause | Comment |
|---|---|
Transient red cell aplasia | Caused by parvovirus B19. Associated with reticulocytopenia |
Increased hemolysis | Often accompanies an acute crisis (painful crisis, ACS, girdle syndrome etc.) |
Acute splenic sequestration | Splenomegaly, abdominal pain, most common in children |
Acute hepatic sequestration | Acute hepatomegaly and abdominal pain |
Delayed hemolytic transfusion reaction or hyperhemolysis | Recent history of transfusion (See Chapter 18)
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