Page 220 - Essential Haematology

206 / Chapter 15 Myeloproliferative neoplasms

(a) (b)

Figure 15.5 (a) The feet of a 72 - year - old man with polycythaemia rubra vera. There is infl ammation of the right

metatarsophalangeal and other joints caused by uric acid deposits. (b) Gangrene of the left fourth toe in
essential thrombocythaemia.

Figure 15.6 Iliac crest trephine

biopsies. (a) Polycythaemia vera:
fat spaces are almost completely
replaced by hyperplastic
haemopoietic tissue. All
haemopoietic cell lines are
increased with megakaryocytes
particularly prominent. (b) Primary
myelo fi brosis: normal marrow
architecture is lost and
haemopoietic cells are
surrounded by increased fi brous
(a) (b) tissue and intercellular substance.

Treatment red cell volume is required (e.g. at the start of
therapy). It is especially indicated in younger patients
Treatment is aimed at maintaining a normal
and those with mild disease. The resulting iron defi -

blood count. The haematocrit should be main-

ciency may limit erythropoiesis. Unfortunately, ven-
tained at about 0.45 and the platelet count below esection does not control the platelet count.
9
400 × 10 /L.
Venesection Cytotoxic m yelosuppression
Venesection to reduce the haematocrit to less than Th is is considered if there is poor tolerance of ven-
0.45 is particularly useful when a rapid reduction of esection, symptomatic or progressive splenomegaly,