Haematologic malignancy AB Flashcards
Thymoma - what are the associations?
Myasthenia gravis
Red cell aplasia
Dermatomyositis
Also SLE, SIADH
What is the most common subtype of Hodgkin lymphoma?
Nodular sclerosing
What are the most common types of transformations seen in patients with polycythaemia rubra vera?
Myelofibrosis or AML (15%)
Burkitt’s lymphoma is associated with a mutation in gene?
t(8;14)
MYC oncogene
Mantle cell lymphoma is associated with a mutation in gene?
t(11;14)
Deregulation of the cyclin D1 (BCL-1) gene
What are the classical features of hyperviscosity syndrome?
Visual changes Bleeding Neurological symptoms (vertigo, hearing loss, ataxia, headaches, etc)
How does Waldenström’s macroglobulinaemia typically present?
Hyperviscosity syndrome (30%) Hepatosplenomegaly Lymphadenopathy Constitutional symptoms Mucosal bleeding (platelet dysfunction) Sensorimotor neuropathy
Which antibody typically causes peripheral neuropathy in Waldenström’s macroglobulinaemia?
Anti–myelin-associated glycoprotein (anti-MAG) Ab
What are the complications of Waldenström’s macroglobulinaemia?
Cryoglobulinaemia (type 1 and 2) Peripheral neuropathy (anti-MAG associated) Cold agglutinin haemolytic anaemia ITP Schnitzler syndrome
Rare: acquired C1 esterase deficiency, vWD
What is Schnitzler syndrome?
Chronic urticarial rash Monoclonal gammopathy Intermittent fevers Arthralgia or arthritis, bone pain Lymphadenopathy
What occurs in ATRA syndrome?
Release of CK
Decreased sats/ARDS
Rx: Dexamethasone
What are 2 cancer syndromes associated with haematologic malignancy?
Li Fraumeni (p53) Bloom syndrome (BLM gene, DNA helicase)
What are two heritable mutations in haematologic malignancy?
Runx1 - acute leukaemia
GATA2 - myelodysplasia
Which haematologic malignancies are associated with EBV?
Hodgkin lymphoma
Burkitt lymphoma
PTLD
Which haematologic malignancies are associated with HTLV-1?
Acute T-lymphoblastic leukaemia/lymphoma
