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Hematology Flashcards

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1
Q

what is a malignancy affecting the differentiated b cells in the bone marrow (plasma cells)

A

myeloma

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2
Q

what is a multiple myeloma

A

neoplastic monoclonal proliferation of plasma cell
myeloma affects multiple bone marrow areas in the body

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3
Q

in what condition do you find bence jones protein in urine

A

myeloma

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4
Q

pathophysiology of myeloma

A

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

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5
Q

symptoms of myeloma

A

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

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6
Q

investigations for myeloma

A

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!

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7
Q

differential for hematology

A

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

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8
Q

treatment for myelomas

A

chemotherapy, bisphosphates (ends in dronate), dialysis

consider bm stem cell transplant

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9
Q

whats an indolent lymphoma and give 3 subtypes

A

slow growing and advanced at presentation
incurable, death around 10 years later
follicular lymphoma
mantle cell lymphoma
CLL

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10
Q

what are the 2 types of lymphomas

A

hodgkins (speficic disease) and non hodgkins (the rest)

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11
Q

where do you find reed sternberg cells

A

Hodgkin’s lymphoma

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12
Q

causes of lymphomas

A

primary immunodeficiencies
secondary immunodeficiency- hiv, recipients of transplants
infection- ebv, helicobacter pylori
autoimmune disorders

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13
Q

general symptoms of lymphomas

A
  • lymphadoenopathy
  • b symptoms- fevers, drenching night sweat, appeptie loss
  • SVC obstruction
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14
Q

what are b symptoms you find in lymphomas

A

fever
unintentional weight loss
night sweat

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15
Q

side effects of chemo treatment for lymphomas

A

alopecia, infertility, nausea

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16
Q

what classification system is used for lymphomas + outline it

A

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
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17
Q

epidemiology of Hodgkins lymphoma

A
  • young adults- peaks in teens and early twenties, again after 70
  • previous epstein bar virus
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18
Q

presentation of Hodgkins lymphoma

A
  • b symtoms- fever, weightloss, night sweats
  • painless rubbery lymphadenopathy - WORSE AFTER DRINKING ALCOHOL
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19
Q

what are the investigations for hodgking lymphoma

A

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

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20
Q

in lymph node biopsy for Hodgkins lymphoma what is the diagnostic feature to look out for

A

reed sternberg cells

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21
Q

treatment for Hodgekins lymphoma

A

chemo + radiotherapy

ABVD chemo

  • adriamycin
  • bleomycin
  • vindlastine
  • dacarbazine

(se= infertility)

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22
Q

presention of Non Hodgkins lymphoma

A

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

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23
Q

investigation for Non hodgkins lymphoma

A

lymph node biopsy- diagnostic- NO reed sternberg cells or popcorn cell

CT/ MRI chest, abdo, pelvis for staging

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24
Q

treatment for Non hodgkins lymphoma

A

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
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25
malignant clonal expansion of lymphocytes which occur at different stages of lymphocyte development- whats this
non hodgkins lymphoma
26
absence of reed sternberg cells- what does this show
non hodgkins lymphoma
27
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
28
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
29
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
30
down syndrome is associated most with what type of leukemia, also in children less than 6
acute LYMPHOID leukmia
31
what's the difference between cells in acute and chronic leukaemia
acute- cells don't differentiate at all chronic- cells partially differentiate
32
what is cytopenia
low levels of rbc or wbc or platelets (anaemia, leukopenia, thrombocytopenia)
33
definition of acute myeloid leukemia
proliferation of myeloblasts- -so no basophil, neutrophil and eosinophil
34
risk factors for acute myeloid leukemia
radiation, chemo
35
presentation of acute myeloid leukemia
bone marrow failure- - neutropenia- less neutrophils - anaemia, - low hb - thrombocytopenia - less platelet SWOLLEN GUMS splenomegaly
36
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
37
treatment for acute myeloid leukemia
supportive treatment correcting anaemia, thromboctyopenia chemo + allopurinol- prevent tumor lysis syndrome
38
definition of chronic myeloid leukemia
overproduction of myeloid progenitor- which comes before the myeloblast
39
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
40
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
41
investigation of chronic myeloid leukemia
genetic testing for BCR ABL - philidelphia chromosome bone marrow biopsy from iliac crest- shows increase in myeloid progenitors fbc
42
treatment of chronic myeloid leukemia
IMATINIB- inhibits tyrosine kinase
43
definition of acute lymphoblastic leukemia
rapid proliferation of lymphoblasts
44
risk factors/ epidemiology of acute lymphoblastic leukemia
children under 6 downs syndrome radiation
45
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
46
investigation inc diagnostic of acute lymphoblastic leukemia
bone marrow biopsy- >20% lymphoblasts (diagnostic) blood film- increased lymphoblasts
47
treatment of acute lymphoblastic leukemia
chemo
48
what is chronic lymphoblastic leukemia
failure of cell apoptosis, most common leukemia overall
49
presentation of chronic lymphoblastic leukemia
asymptomatic lymphadenopathy and bone marrow failure
50
investigation inc diagnostic of chronic lymphoblastic leukemia
diagnostic= blood film- smudge cells!
51
treatment for chronic lymphoblastic leukemia
chemo, pallitative if old
52
complication of chronic lymphoblastic leukemia
richters transformation- b cells massively accumulate in lymph nodes- massive lympadenopathy and transformation from cll— agressive lymphoma
53
size of microcytic anemia
<80fcv
54
what is the size of anemia based on
mean copuscular volume- average size of rbc
55
types of microcytic anemia
thalassaemia (hemolytic) anemia of chronic disease iron deficiency lead poisoning sideroblastic
56
where is iron absorbed
duodenum and upper jejunum
57
what is iron bound to to travel
transferrin
58
what is iron stored as
ferritin
59
features of anemia
- Peripheral coldness - conjuctival pallor - tachycardia - fatigue`` - brittle hair - exertional dyspnoea
60
what protein facilitates fe absorbtion
transferrin
61
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
62
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
63
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
64
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
65
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
66
thalassemia- is it genetic?
yes- autosomal recessive , genetic defect in the protein chain that makes up hb
67
where is thalassaemia prevelant
where malaria is- south asia, india, middle east
68
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
69
presentation of thalassaemia
chimponk face- massively enlarged forhead and cheekbones hepatospleenomegaly ruq pain gallstones failure to thrive may get jaundiced
70
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
71
treatment for thalassaemia
regular blood transfusion + iron chelton therapy splenectomy + folate supplements + bm stem cell transplant
72
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
73
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
74
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
75

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