Haematology Flashcards
What is Polycythemia Vera?
A myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets.
A mutation in JAK2 is present in 95% of patients
commonly presents in 60s
Features of Polycythaemia Vera
hyperviscosity pruritus, typically after a hot bath splenomegaly haemorrhage (secondary to abnormal platelet function) plethoric appearance hypertension in a third of patients low ESR isolated raise in haemoglobin
Investigations for Polycythaemia vera
full blood count/film (raised haematocrit; neutrophils, basophils, platelets raised in half of patients)
JAK2 mutation
serum ferritin
renal and liver function tests
blood film in Hyposplenism e.g. post-splenectomy
target cells Howell-Jolly bodies Pappenheimer bodies siderotic granules acanthocytes
blood film in Iron-deficiency anaemia
target cells
‘pencil’ poikilocytes
if combined with B12/folate deficiency a ‘dimorphic’ film occurs with mixed microcytic and macrocytic cells
blood film in myelofibrosis
‘tear-drop’ poikilocytes
blood film in intravascular haemolysis
schistocytes
Blood film in megaloblastic anaemia
hypersegmented neutrophils
What is Myelofibrosis?
a myeloproliferative disorder
hyperplasia of abnormal megakaryocytes with resultant release of platelet-derived growth factor stimulates fibroblasts
haematopoiesis develops in the liver and spleen
Features of Myelofibrosis
e.g. elderly person with symptoms of anaemia e.g. fatigue (the most common presenting symptom)
massive splenomegaly
hypermetabolic symptoms: weight loss, night sweats etc
Laboratory findings in Myelofibrosis
anaemia
high WBC and platelet count early in the disease
‘tear-drop’ poikilocytes on blood film
unobtainable bone marrow biopsy - ‘dry tap’ therefore trephine biopsy needed
high urate and LDH (reflect increased cell turnover)
What is Post thrombotic syndrome?
features (5)
Management
complications following a DVT. Venous outflow obstruction and venous insufficiency result in chronic venous hypertension. Features: painful, heavy calves pruritus swelling varicose veins venous ulceration
compression stockings and elevation
What is Hodgkins Lymphoma?
A malignant proliferation of lymphocytes characterised by the presence of the Reed-Sternberg cell. It has a bimodal age distributions being most common in the third and seventh decades
What is Nodular sclerosing Hodgkins lymphoma?
Most common (around 70%)
Good prognosis
More common in women
Associated with lacunar cells
What is Mixed cellularity Hodgkins lymphoma?
Around 20%
Good prognosis
Associated with a large number of Reed-Sternberg cells
