HAEM - Leucocytes and neutrophils Flashcards
Fct neutrophils
Ingest and kill bacterial, fungal and cellular debris
Fct lymphocyte s
Produce antibodies for cell mediated immunity
Fct eosinophils
Role in allergic reactions
Defense of parasitic infection
Fct monocytes
Precursor tissue macrophages
Fct basophil
Release histamine in inflammatory reactions
Role spleen (5)
Reservoir of RBC
Site of B lymphocyte + antibody production
Reservoir platelets
Reservoir macrophages
Removes antibody coated bacteria and blood cells
Neutrophilia DDx >10 (5)
bacterial infection Tissue damage - MI.PE, burn Physiological - exercise, during preg CCS + inflamm - Gout, RA, UC, Chron's Malig - CML/solid tumour
Neutropenia DDx <1.5 (7)
Viral infection + severe bacterial infection - sepsis /typhoid Autoimmunity - commonly SLE Felty's syndrome Black people Alcohol + other drugs. Methotrexate ELA2 gene mutation BM failure
Neutropenic sepsis
Severe neutropenia leading to sepsis
Agranulocytosis
Complete abscence of circulating neutrophils
Which med is agranulocytosis a common SE of?
Carbimazole
Lymphocytosis DDx >5 (4)
Viral infections
Chronic infections - TB/toxoplasmosis/syphillis
Lymphoproliferative - CLL/some lymphomas
Post splenectomy
Lymphopenia DDx (5)
HIV/autoimmunity CCS + inflamm - CT disease Chemotherpy + lymphoma Renaal failure Transient - recent infection
Hx points - abnormal WCC (8)
Age Duration Sx W loss >10% 6m Drenching night sweats Unexplained fever Systemic disease - RA Evidence malig Lymphadenopathy >6w + >2cm
Possible Ix - abnormal WCC (6)
Blood film Immunophenotyping USS/CT Microbiology LN biopsy BM biopsy
What are the 2 lineages a multipotent cell can go down
Common myeloid progenitor
Common lymphoid progenitor
Cells the common myeloid progenitor can DDx into (5)
Erythrocyte Mast cell Megakaryocytes Neutrophil MOnocyte
What do megakaryocytes further divide into
Platelets
What do common lymphoid progenitor cells DDx into
B cell
T cell
NK cell
What is immature cells in peripheral blood a sign of?
That the marrow is stressed
Hence pre-leukaemic
ALL
Malignancy of lymphoid cells of B/T lineage –> uncontrolled proliferation of immature blast cells, BM failure and tissue infiltration
Who gets ALL
Children - 3-7y/o
Which syndrome is ALL more common in?
Downs
Prognosis ALL
80% cure rate in kids <10
Indicators - poor prognosis ALL (4)
Older ps
Male
B cell disease
Philadelphia chromosome
Philadelphia chromosome
9:22
What is AML
Malignancy of blast cells fom marrow myeloid elements
RF AML (4)
Myeloproliferative disorders
Previous chemotherapy
IR
Genetic syndromes
Median age PS AML
65
Prognosis AML
20% 3 y survival after chemo
Sx - acute leukaemia (6)
B Sx Bone pain Anaemia --> SOB on exertion, weakness Leukopenia --> rec infections Thrombocytopenia --> bleeding + bruising HS megaly
Ix Acute leukaemia
FBC Blood film - BLASTS CXR BM aspiration PET scan - mets U/E, LFT, cardiac fct
What is diagnostic Ix result for acute leukaemia
Blast cells on blood film
ALL - CXR findings
Mediastinum widening
General supportive management - acute leukaemia
Nurse Hickman line High kcal diet Freq blood + platelet transfusion Allopurinol Check blood/obs - signs infections
Use of ABx in acute leukaemias
If T>38’ on 2 occasions >1hr
Cephalosporin + gentamicin until afebrile 72hrs
Why give allopurinal to patients with acute leukaemia
To prevent tumour lysis syndrome due to chemo
Specific Mx ALL
High dose chemo
+ 2 y maintenance therapy
Consider transplant if poor prog/relapse
ALL cure if have Philadelphia chromosome
Transplant (BM)
Specific Mx AML
Intensive chemo
If poor prog AML - what Mx should be done
Allogenic marrow transplant after 1st round chemo
If intermediate prod AML - what Mx should be done
Autologous marrow transplant
Age - CML
40-60y/o
Which genetic linkage accounts for 95% cases CML
Philadelphia chromo
Sx CML
B Sx
Gout + abdo discomfort
O/E CML
Massive HS megaly
Signs anaemia /thrombocytopenia
Ix CML
FBC - WCC v high
Blood film
BM
CT/PET
Blood film findings - CML
Spectrum myeloid cells
BM findings - CML
Hypocellular
Mx CML
Imantinib chemo
SC transplant
Prognosis CML
Median survival 6y
What are the 3 stages of CML
Chronic - few Sx for y
Accelerated- incr Sx
Blast transformation - features acute leukaemia + death
What is the most common leukaemia
CLL
M:F CLL
2:1
Median age of PS - CLL
70 y/o
Pathophysiology - CLL
Accumulation of mature B cells that have escaped apoptosis
Increased mass of immune competent cells –> BM failure
Sx CLL
Often none
Anaemic/infection prone
O/E CLL (2)
Large non-tender LN
HSmegaly
Ix CLL
FBC - raised lymphocytes
Blood film
Blood film results CLL
Smudge cells
Mx CLL
Symptomatic Tx only
chemo
Radiotherapy for HS megaly
How is prognosis of CLL determined
Rai stage
Rai stage 0 + prognosis
Lymphocytosis alone
> 13y
Rai stage 1 + prognosis
Lymphocytosis + lymphadenopathy
8 y
Rai stage 2 + prognosis
Lymphocytosis + HSmegaly
5 y
Rai stage 3 + prognosis
Lymphocytosis + anaemia
2 y
Rai stage 4 + prognosis
Lymphocytosis + thrombocytopenia
1y
What is multiple myeloma
Malignant clonal proliferation of plasma cells . Single clone of plasma cells –> single Ig –> monoclonal band
Av age PS Multiple myeloma
70
Which population have increased prevelance of multiple myeloma
Afro-Carribbean
PS Multiple myeloma
Osteolytic bone lesions –> backache, patho fractures = hypercalcaemia
BM failure –> infections, Sx anaemia + bleeding
Renal impairment
Ix Multiple myeloma
FBC Blood film ESR - raised U/E - deranged Ca - raised ALP - norm Electrophoresis - paraprotein MC band Urine Bence Jones protein +ve Skeletal XR BM biopsy >10% clonal plasma cells
Blood film finding multiple myeloma
Rouleaux formation
FBC finding multiple myeloma
Normochromic normocytic anaemia
Leukopenia
Skeletal XR finding multiple myeloma
Pepper pot skull
If BM biopsy in Multiple myeloma yields <10% plasma clone cells, what is the diagnosis
MGUS
Mx multiple myeloma
Supportive
Chemo/radiotherapy
BM SC transplant if <70
Prognosis multiple myeloma
3-4y
What is lymphoma?
Malignant proliferation of lymphocytes
% lymphoma which are Hodgkins lymphoma
15%
What are Hodgkins lymphoma defining characteristic
Reed-Sternburg cells
Age range Hodgkin’s lymphoma
20-35
RF Hodgkin’s lymphoma (4)
Affected sibling
EBV
SLE
Obesity
Sx Hodgkin’s lymphoma (3)
Enlarged, non-tender rubbery LN
B Dx
Mass effects/direct extension Sx from mediastinal LN
What can induce LN pain in pt with Hodgkin’s lymphoma?
Alcohol
O/E - Hodgkin’s lymphoma (4)
Lymphadenopathy
Anaemia
Cahexia
HS megaly (50%)
How is Non-Hodgkin’s lymphoma different from Hodgkins
W/o presence Reed-Sternburg cells
Peak age Non-Hodgkin’s lymphoma
70
High grade Non-Hodgkin’s lymphoma
Divide rapidly
PPS rapid onset lymphadenopathy
> aggressive
Better prognosis if ID’d
Low grade Non-Hodgkin’s lymphoma
Divide slowly
> Insidious
Widely disseminated at diagnosis + incurable
Sx Non-Hodgkin’s lymphoma (4)
Superficial lymphadenopathy (75%)
Extranodal Sx - oropharynx, skin, CNS, gut, lung
B Sx
BM failure
Ix Non-Hodgkin’s lymphoma (6)
FBC, ESR, U+E Ca2+ Film LDH LN excision biopsy Staging CT
Mx Non-Hodgkin’s lymphoma
Chemo/radiotherapy
Or
Chemo-radiotherapy
Poor prognostic factors non-hodgkin’s (3)
> 60
Disseminated disease
Raised LDH
Staging for lymphomas
Ann Arbor
What is meant by myeloproliferative disorders
Clones of SC proliferate in BM
yet retain ability to differentiate
What is essential thrombocytosis
Clonal proliferation of megacaryocytes –> persistently raised platelets
Sx essential thrombocytosis
Often astmp
Microvascular occlusion
Bleeding
aa/vv thrombosis
What is polycythaemia rubra vera
Malig proliferation of clone derived from 1 pluripotent marrow cell –> erythroid progenitor offspring which don’t req EPO to avoid apoptosis
Consequence of PCV on the blood
XS prod RBC, WBC and platelets –> hyperviscosity + thrombotic complications
Diagnosis PCV (2)
Incr red cell mass
JAK2 mutation
PS PCV
Thrombosis rarer: hyperviscocity Sx: headache, dizzy, tinnitus, facial plethora, erythromelalgia Splenomegaly Gout
Tx PCV
Venesection
Low dose aspirin
How to DDx 1’ and 2’ PCV
In 2’ (e.g. b.c hypoxia//renal disease), only RBC count is raised
What is primary myelofibrosis
Hyperplasia of megakaryocytes –> XS platelet derv’d GF –> marrow fibrosis + metaplasia
Sx primary myelofibrosis
B Sx
Abdo discomfort (from massive HS megaly)
BM failure
What is aplastic anaemia
Rare SC disorder –> pancytopenia and hypoplastic marrow
Cause aplastic anaemia
Autoimmune - Dx, viruses, irradiation
Fanconi syndrome
Sx aplastic anaemia
BM failure
Diagnosis aplastic anaemia
BM biopsy
Mx aplastic anaemia
Blood product transfusion
Immunosuppression
extra Tx aplastic anaemia - younger pt
Allogenic BM transplant may cure