Exam 2- white blood cells Flashcards
How is lymph returned to the circulation? (left and right)
L- at the junction of the left subclavian and jugular veins, from the thoracic duct which drains most of the body;
R- at the junction of the right subclavian and jugular veins, from the right upper limb, head and neck
What does generalised lymphadenopathy suggest?
A systemic inflammatory process or widespread maligancy e.g. leukaemia, lymphoma
Functions of the spleen (2)
filter for old and damaged red blood cell- removes from circulation
functions like a large lymph node- antigen presentation, lymphoid follicles
What are the features of splenic enlargement? (2)
LUQ discomfort/pain
Hypersplenism
What is the triad of hypersplenism? Generally what causes hypersplenism?
Cytopenia [1] which is corrected by splenectomy [2]; splenomegaly [3];
Anything which causes splenomegaly can cause hypersplenism
Causes of splenomegaly? (5)
Infection e.g. infectious mononucleosis Haemolysis e.g. hereditary spherocytosis Congestive e.g. portal hypertension, cardiac failure Infiltrative e.g. sarcoidosis Neoplastic e.g. leukaemia, lymphoma
Nuclear remnants in red blood cells; feature of hyposplenism
Howell-Jolly bodies
What is acute lymphoblastic leukaemia (ALL) and which group of patients is it commonest in?
Uncontrolled proliferation of B cell/T celll blasts;
Commonest cancer of childhood, rare in adults
Signs and symptoms of acute leukaemia?
a) marrow failure (3)
b) infiltrative (4)
a) anaemia, infection, bleeding
b) hepatosplenomegaly, lymphadenopathy, gum hypertrophy, CNS infiltration leading to cranial nerve palsies (ALL)
What is acute myeloid leukaemia? (AML)
What is AML sometimes a complication of?
Proliferation of myeloid blast cells;
commoner in adults; sometimes a complication of chemotherapy
Treatment of leukaemias in general (3)
Intensive chemotherapy
Supportive care e.g. blood, platelets, fluids (insert Hickman line)
Allogeneic bone marrow transplants
Investigations where acute leuakamia is suspected (4)
Blood count and film
Bone marrow aspirate + immunophenotyping, cytogenic analysis
What complication of chemotherapy can result in acute renal failure? How can this be prevented?
Tumour lysis syndrome.
Allopurinol + IV fluids
What pattern of metabolic abnormalities in seen in tumour lysis syndrome? (4)
Hyper- kalaemia, phosphataemia, uricaemia
Hypocalcaemia
Fever + fatigue + splenomegaly + t (9,22)
Chronic myeloid leukaemia
t(9,22) creates what fusion protein?
creates a fusion Bcr-Abl protein
How is CML treated? (2)
Imatinib- Brc-Abl tyrosine kinase inhbitor
Stem cell tranplantation
What is the natural history of CML? (3)
Chronic (months -> years, few symptoms)
Accelerated (increasing spleen size and symptoms)
Blast transformation- features of acute leukaemia and death
Vague symptoms + peripheral blood lymphocytosis (mature lymphocytes)
Chronic lymphocytic leukaemia
Natural history of CLL (3). What is often the cause of death in CLL?
1/3 never progress
1/3 progress in time
1/3 actively progressing
Infection, or transformation to aggresive lymphoma
What is lymphoma and how is it classified?
Malignant proliferations of lymphocytes, which accumulate in the lymph nodes, but also peripheral blood/organs.
Hodgkins and non-Hodgkins (T cell and B-cell)
General symptoms of Hodgkins lymphoma? (3)
What are the B-symptoms and what do they indicate? (3)
Painless rubbery enlarged lymph nodes (typically cervical)
Pruritus
Alcohol-induced lymph node pain
“B-symptoms”- indicates advanced disease:
Night sweats
Unexplained fever
Weight loss
Cells with “mirror-image nuclei” characteristic of Hodgkins lymphoma
Reed-Sternberg cells
What is the difference between stage II and stage III Hodgkins lymphoma?
Stage III involves lymph nodes on both sides of the diaphragm
malignant clonal proliferation of plasma cells
Myeloma
Osteolysis, hypercalcaemia, features of marrow failure, renal impairment- diagnosis?
Myeloma
Tests in myeloma:
a) blood film
b) urine
c) serum
d) skeletal survey
a) Rouleaux formation on blood film
b) Bence-Jones proteins (free light chains)
c) Monoclonal band in serum electrophoresis; elevated urea and creatinine
d) osteolytic lesions
Treatment of myeloma (4)
Chemo
steroids
thalidomide
autologous stem cell transplant
Slowly progressive multisystem disease caused by deposition of insoluble light chains in various organs
AL amyloidosis
Diagnostic test for AL amyloidosis?
Biopsy- Congo red stain- apple-green birefringence under polarised light
Lymphoplasmacytoid neoplasm causing a characteristic IgM paraprotein
Waldenstroms
Manifestations of waldenstroms (4)
Hyperviscosity syndrome
Neuropathy
Splenomegaly, lymphadenopathy
Marrow failure
Structure of antibody?
Two heavy chains and two light chains with variable region generated by V-D-J recombination
What is the main difference between myeloproliferative disorders and leukaemia?
Maturation is relatively preserved in MPD
Classification of myeloproliferative disorders:
a) red cell
b) white cells
c) platelets
d) fibroblasts
a) polycythaemia rubra vera
b) chronic myeloid leukaemia
c) thrombocythaemia
d) myelofibrosis
Causes of secondary polycythaemia (3)
Chronic hypoxia e.g. COPD
smoking
EPO-secreting tumour
JAK2 mutation is present in 90% of patients with what disease?
Polycythaemia rubra vera
Splenomegaly and neutropenia in a patient with rheumatoid arthritis?
Felty syndrome
Commonest myeloma subtypes?
IgG (2/3rds)
IgA (1/3rd)
High-grade B cell lymphoma associated with EBV, endemic in Africa
Burkitt’s lymphoma
