Hematology Flashcards
what is a malignancy affecting the differentiated b cells in the bone marrow (plasma cells)
myeloma
what is a multiple myeloma
neoplastic monoclonal proliferation of plasma cell
myeloma affects multiple bone marrow areas in the body
in what condition do you find bence jones protein in urine
myeloma
pathophysiology of myeloma
clonal proliferation of plasma cells
normally- plasma cells produce a wide range of igs, but in myeloma, malignant plasma cells produce an excess of one type of ig- paraprotein- usually igG and A
symptoms of myeloma
C- calcium raised (hypercalcemia symptoms due to increased ostecvlast bone resoption - bones, stones, abdo moans, pyshciatric grans
R- renal failure- hypercalcemia can cause renal stones, mainly - immunoglobulin light chain kappa can deposit in organs like kidneys- you get BENCE JONES PROTEIN IN PEE
A- anemia
B- bone leisions- increased osteoclastic activity causes boneache- backache is the most common syndrome
investigations for myeloma
fbc+ blood film- anemia, esr increase, + rouleaux formation
urine dipstick- BENCE JONES PROTEIN
u+e= renal failure
serum protein electropheresis: ig prarprotein M spike- hypergammaglobuliemia for that specific IG
xr of skull- pepper pot skull
osteolytic lesions of punchedout holes
BM biopsy- >10% plasma cells!
differential for hematology
MGUS- mammyloid gammopathy of undetermined significance- this is a precursor to myeloma but not myeloma itself. this is asymptomatic, has little paraprotein spile and <10% BM plasma cells
treatment for myelomas
chemotherapy, bisphosphates (ends in dronate), dialysis
consider bm stem cell transplant
whats an indolent lymphoma and give 3 subtypes
slow growing and advanced at presentation
incurable, death around 10 years later
follicular lymphoma
mantle cell lymphoma
CLL
what are the 2 types of lymphomas
hodgkins (speficic disease) and non hodgkins (the rest)
where do you find reed sternberg cells
Hodgkin’s lymphoma
causes of lymphomas
primary immunodeficiencies
secondary immunodeficiency- hiv, recipients of transplants
infection- ebv, helicobacter pylori
autoimmune disorders
general symptoms of lymphomas
- lymphadoenopathy
- b symptoms- fevers, drenching night sweat, appeptie loss
- SVC obstruction
what are b symptoms you find in lymphomas
fever
unintentional weight loss
night sweat
side effects of chemo treatment for lymphomas
alopecia, infertility, nausea
what classification system is used for lymphomas + outline it
Lugano classification
emphasises weather the affected nodes are above or below the diaphragm
- stage1- one node/ group of nodes
- 2- >1 groupd of nodes but same side of diaphragm
- 3- lymph nodes both above and below diaphragm
- 4- widespread involvement including non lymphatic organs like lungs or liver
epidemiology of Hodgkins lymphoma
- young adults- peaks in teens and early twenties, again after 70
- previous epstein bar virus
presentation of Hodgkins lymphoma
- b symtoms- fever, weightloss, night sweats
- painless rubbery lymphadenopathy - WORSE AFTER DRINKING ALCOHOL
what are the investigations for hodgking lymphoma
lymph node biopsy!!- look for reed sternberg cells (diagnostic)
- a variant- nodular lymphocyte predominant Hodgkin’s produces POPCORN CELLS
blood test- high esr, high lactasse degydrogenase (ldh), low hb
use ct/mri for chest/ abdo/ pelvis for staging
in lymph node biopsy for Hodgkins lymphoma what is the diagnostic feature to look out for
reed sternberg cells
treatment for Hodgekins lymphoma
chemo + radiotherapy
ABVD chemo
- adriamycin
- bleomycin
- vindlastine
- dacarbazine
(se= infertility)
presention of Non Hodgkins lymphoma
b symptoms- fever, weeight loss, drenching night sweats
painless rubbery lypadenopathy- NOT affected by alcohol
more varied and will affect more parts of the body
investigation for Non hodgkins lymphoma
lymph node biopsy- diagnostic- NO reed sternberg cells or popcorn cell
CT/ MRI chest, abdo, pelvis for staging
treatment for Non hodgkins lymphoma
depends on subtype- indolent/ low grade= watch and wait, alkylyting agents, combo chemo, radio
aggresive/ high grade:
chemotherapy
R-CHOP
- Rituximab
- Cyclophosphamide
- Hydroxy-daunorubicin
- O – Vincristine (oncovin is brand name)
- Prednisolone
malignant clonal expansion of lymphocytes which occur at different stages of lymphocyte development- whats this
non hodgkins lymphoma
absence of reed sternberg cells- what does this show
non hodgkins lymphoma
what is myeolodysplastic syndrome and what can it transform into
form of cancer from mutation of myeloid cells in bone marrow- they now do inaffective haematopoesis- turn into myeloid leukaemia
what blood conditions does myelodysplastic syndrome cause
causes low level of blood components that originate from myeloid cell line
- anaemia - low hb
- neutropenia- low neutrophil count
- thrombocytopenia- low platelets
types of leukemia
-acute myeloid leukemia- rapid progression cancer of myeloid cell line
- chromic myeloid leukaemia- slow progresion
- acute lymphoblastic leukaemia- rapid progression of cancer of lymphoid cell line
- chronic lymphoblast leukemia- slow progression of above
down syndrome is associated most with what type of leukemia, also in children less than 6
acute LYMPHOID leukmia
what’s the difference between cells in acute and chronic leukaemia
acute- cells don’t differentiate at all
chronic- cells partially differentiate
what is cytopenia
low levels of rbc or wbc or platelets (anaemia, leukopenia, thrombocytopenia)
definition of acute myeloid leukemia
proliferation of myeloblasts- -so no basophil, neutrophil and eosinophil
risk factors for acute myeloid leukemia
radiation, chemo
presentation of acute myeloid leukemia
bone marrow failure-
- neutropenia- less neutrophils
- anaemia, - low hb
- thrombocytopenia - less platelet
SWOLLEN GUMS
splenomegaly
investigation for acute myeloid leukemia
fbc+ blood film- pancytopenia- deficiency of rbc, wbc, platelets
- neutropenia
- thrombocutopenia
- anemia
cytology- AURER RODS (cytoplasmic aggregagte)
GS= bone marrow biopsy- >20% myeloid blasts
treatment for acute myeloid leukemia
supportive treatment correcting anaemia, thromboctyopenia
chemo + allopurinol- prevent tumor lysis syndrome
definition of chronic myeloid leukemia
overproduction of myeloid progenitor- which comes before the myeloblast
pathophysiology of chronic myeloid leukemia
mutation in philidelphia chromosone- activation of tyrosine kinase- granulocytes divide too quickly- too many premature cells- accumulate in bone marrow
presentation of chronic myeloid leukemia
MASSIVE SPLENOMEGALY
hyperviscosity- headaches, thrombotic events,
bone marrow faulure- neutropenia, anaemia, thrombocytopenia
b symptoms- wieght loss, night sweats, loss of apetite
investigation of chronic myeloid leukemia
genetic testing for BCR ABL - philidelphia chromosome
bone marrow biopsy from iliac crest- shows increase in myeloid progenitors
fbc
treatment of chronic myeloid leukemia
IMATINIB- inhibits tyrosine kinase
definition of acute lymphoblastic leukemia
rapid proliferation of lymphoblasts
risk factors/ epidemiology of acute lymphoblastic leukemia
children under 6
downs syndrome
radiation
presentation of acute lymphoblastic leukemia
bone marrow faulure-
- anaemia: lethargy and pallor
- neutropaenia: frequent or severe infections
- thrombocytopenia: easy bruising, petechiae
b symptoms- more common than aml
hepatosplenomegaly
SWOLLEN TESTICLES
investigation inc diagnostic of acute lymphoblastic leukemia
bone marrow biopsy- >20% lymphoblasts (diagnostic)
blood film- increased lymphoblasts
treatment of acute lymphoblastic leukemia
chemo
what is chronic lymphoblastic leukemia
failure of cell apoptosis,
most common leukemia overall
presentation of chronic lymphoblastic leukemia
asymptomatic
lymphadenopathy and bone marrow failure
investigation inc diagnostic of chronic lymphoblastic leukemia
diagnostic= blood film- smudge cells!
treatment for chronic lymphoblastic leukemia
chemo, pallitative if old
complication of chronic lymphoblastic leukemia
richters transformation- b cells massively accumulate in lymph nodes- massive lympadenopathy and transformation from cll— agressive lymphoma
size of microcytic anemia
<80fcv
what is the size of anemia based on
mean copuscular volume- average size of rbc
types of microcytic anemia
thalassaemia (hemolytic)
anemia of chronic disease
iron deficiency
lead poisoning
sideroblastic
where is iron absorbed
duodenum and upper jejunum
what is iron bound to to travel
transferrin
what is iron stored as
ferritin
features of anemia
- Peripheral coldness
- conjuctival pallor
- tachycardia
- fatigue``
- brittle hair
- exertional dyspnoea
what protein facilitates fe absorbtion
transferrin
causes of iron deficiency anemia
diet/ malnutrition= not enough red meat/ beans- vegan?
malabsorptibe conditions- coeliac, ibd
bleeding- colorectal cancer, menstruation
pregnancy- a lot os transferred to foetus
kids- increased requirement
pathophysiology of iron deficiency anemia
- fe is usually absorbed and circulates bound to transferrin, stored as ferritin or incorporated into heamoglobin
- in iron deficiency- less iron available for haem synthesis- so less haemoglobin- so lack of effective rbc- so anemia
spefici signs and symptoms of iron deficiency anemia
symptoms:
- tired
- cold
- palpitations
signs:
- koilonychia - spoon shaped nails
- angular stomatitis- ulceration at mouth corners
- atrophic glossitis- tongue enlarged
investigations for iron deficiency anemia
fbc and blood film- microcytic anemia
fe studies- low serum fe, low ferritin, low transferrin saturation, high TIBC
blood film- rbc different size and shapes
reduced reticulocyte count
treatment of iron deficiency anemia and se of it
oral ferrous sulphate- side effect of black stool and diarrhea
if poorly treated then consider ferrous gluconate
thalassemia- is it genetic?
yes- autosomal recessive , genetic defect in the protein chain that makes up hb
where is thalassaemia prevelant
where malaria is- south asia, india, middle east
alpha and beta thallasemia have what chromsomes and genes altered
alpha- deficiency in alpha globin gene, deletion on chromosme 16
beta- deficiency of beta globin gene, mutation on chromosome 11
presentation of thalassaemia
chimponk face- massively enlarged forhead and cheekbones
hepatospleenomegaly
ruq pain
gallstones
failure to thrive
may get jaundiced
investigations for thalassaemia
HB electropheresis!! (diagnositc)- see high HbA2 (beta) and Hb H (alpha)
FBC- microcytic anemia
Blood film- recitulocyte count (increased) and hypochromic rbc
treatment for thalassaemia
regular blood transfusion + iron chelton therapy
splenectomy
+ folate supplements
+ bm stem cell transplant
sideroblastic anemia- what is it and who is affected most, what is it caused by, what is it
x linked inherited congenital condition, mainly affecting boys
caused by excess alcohol intake, vitamin B6 deficiency, lead poisoning
lack of mature rbc, and too much iron so irons not used in hb synthesis and stays trapped in mitochondira
build up of iron and low haem
investigation for sideroblastic anemia
FBC AND BLOOD FILM- microcytic with ringed sideroblasts (iron deposits)
FE STUDIES- increased fe, increased ferritin, raised transferrin sats, low TIBC
what IS thalassemia
decreased rate of production or no production of one or more globin in chain in the red cell precurosirs or mature red cells
