Book of Medical Disorders in Pregnancy - Tintash

These patients have long extremities and
may suffer from chronic ulceration of
the legs. Spleen is usually enlarged but
may occasionally be small. Some
patients may even have pelvic
deformities due to softening of the
bones.
Blood picture - The red cells show
normocytic and orthochromic
characteristics. There is constant
presence of reticulocytoses. This may
exceed 30 per cent during the crisis. In
long standing cases the anaemia may
become megaloblastic due to shortage
of folic acid. The red cells may also
show sickling when tested. The
majorities of patients are well adjusted
to their anaemia and remain free from
symptoms with their hemoglobin level
reading as low as 7 or 8 gram per cent.
The sickle cell disease crisis:
The two types generally described are
the hemolytic and painful crisis.
Hemolytic crisis in this the anemia
develops with great rapidity. There is a
polymorphonuclear leukocytosis, and
jaundice. The patient is pyrexial and
fever up to 39.44 Celsius has been
recorded. This type of crisis may be
precipitated by infection anywhere in the
body.
Painful crisis:
This condition is due to sludging of
sickled cells in various organs of the
body, which in turn results in thrombosis
of capillaries and infarctions in the
affected tissues. The pain may be in the
lower part of the back, the long bones
and abdomen. There may be history of
severe vomiting or hematuria.
Hemoptysis can result from pulmonary
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infarction which is caused by fat. Death
may result from heart failure or
infarction during these crises.
Effect on pregnancy:
Homozygous (5-5) Sickle cell Anaemia
- Patients with sickle cell anaemia (5-5)
have low fertility. Somehow this does
not hold true for sickle cell hemoglobin
C disease. There is considerably
increased incidence of intrauterine
growth retardation. The utero decidual
vessels get blocked by sludging and lead
to placental insufficiency. The Sickle
Cell Trait A-S - This has no ill effect on
pregnancy or the foetus.
Effect of disease on pregnancy - Both
hemolytic and painful crises are more
during the last trimester and before
delivery. Hemolytic crisis can be dealt
with repeated blood transfusions.
Diagnosis - Different genetic types of
hemoglobin can be identified by paper
electrophoresis. In sickle cell disease the
two genetic variants of hemoglobin 5
and C, exist together in the red cells and
can also be diagnosed by this method.
The diagnosis must be made on carefully
evaluating the history and clinical features
of the patient. The presence of an
orthochromic and normocytic blood
picture with reduction of the granulocyte
series and platelets should be confirmed.
There should be no evidence of hemolysis.
The hypoplastic bone marrow must
show a diminution in all cellular
elements but there should be no
infiltration of bone marrow with cancer
cells.
Blood picture - In such cases the blood
picture shows orthochromic, normocytic
cells with normal M C H C. and serum