EMQs in Clinical Medicine.pdf - Peshawar Medical College

64 Haematology and oncology
23 Haematological diseases
Answers: B K F I C
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A 25-year-old man presents with enlarged painless lymph nodes in
the neck. His peripheral blood film shows Reed-Sternberg cells.
Reed–Sternberg cells are classically binucleate and pathognomonic of
Hodgkin’s lymphoma. The two large nuclei can give rise to an owl’s eye
appearance.
A 45-year-old man presents with fever, weight loss, tiredness and
gout. On examination there is splenomegaly. White blood cell count is
112 10 9 /l. The Philadelphia chromosome is detected.
Chronic myeloid leukaemia (CML) is a myeloproliferative disorder characterized
by uncontrolled proliferation of myeloid cells where the white cell
count is often 100 10 9 /l. The highest incidence of CML is in middle age.
Myeloproliferative and lymphoproliferative disorders can be associated with
hyperuricaemia and thus gout caused by increased turnover of purines.
The Philadelphia chromosome, a translocation between the long arm of
chromosome 22 to chromosome 9, is seen in around 95 per cent patients.
Treatment options include the use of interferon, hydroxyurea and allogeneic
transplantation from a suitable donor.
A 70-year-old woman complains of weight loss, headache, blurry vision,
lethargy and haematuria. Positive findings on examination include
cervical lymphadenopathy, splenomegaly and numerous retinal haemorrhages.
Bone marrow biopsy shows lymphoplasmacytoid cell infiltrate.
Waldenström’s macroglobulinaemia (WM) is a lymphoplasmacytoid lymphoma
that usually affects older men. The clinical manifestations of the
disease, e.g. hyperviscosity, result from the production of IgM paraprotein.
Myeloma can also give signs of hyperviscosity, but splenomegaly and
lymphadenopathy make WM more likely. Bone marrow biopsy shows the
presence of lymphoplasmacytoid cells. There are usually very few plasma
cells unlike myeloma, which shows a characteristic infiltrate of plasma cells.
A 27-year-old man presents with haemolytic anaemia after surgery.
He reports a history of recurrent abdominal pains. The Ham’s test is
positive.
Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired defect
where red cells are unusually sensitive to destruction by activated complement.
Patients may present non-specifically with recurrent abdominal
pains or with haemolytic anaemia. Venous thrombotic episodes are very
common in patients with PNH. The condition acquired its name from the
characteristic finding of dark-coloured urine when voiding at night or
early in the morning.