haem vol 3 Flashcards
define neutropaeina + signs and symptoms
absolute neutrophil count less than 1.5x10’9.
may be due to decreaced production
or increaced consumption
presents with frequent, prolongued infections which
freq involve skin and mucous membranes, staphlococcal, show minimal clinical features despite severe infection.
when to refer in neutropaenia
if unwell and less than 1x10’9
if presisting more than 1 week and well (1x10’9 <)
1-1.5 needs blood film + B12, folate, ferritin, ANA + a repeat in 4-6 weeks- if persisting then refer.
list some causes of decreaced production and increaced consuption in neutropenia
consumption- sepsis, hypersplenism, autoimmune.
reduced production- marrow aplasia/ infiltration, viral infection, TB, drugs.
investigation of neutropenia
evaluate for acute/ chronic infection
assess spleen size, CBC + reticulocite count.
look at bone marrow- aspiration/ biopsy.
define lymphocytosis, lymphadenopathy and splenomegaly. What linkes them all
lymphocytosis- increace in number of circulating lymphocites >5x10’9 (in adults, 7/ 9 in older/ younger children)
lymphadenopathy- swelling of the lymph nodes, usually 2’ to bacterial or viral infection
splenomegaly- an increace in the volume and size of the spleen to more than 400-500g
all occurs during acute/ chronic infection.
discuss lymphocitosis in more detail, causes, treatment, investigations.
most commonly- viruses (EBV, CMV, HIV)
bacteria (baronella, bordatella)
if monoclonal- chronic lymphocitic leukaemia
non-hodgkin lymphoma.
stress can also cause.
Ix: history is important
B-symptoms- think clonal issues
splenectomy- common after this.
CBC (anaemia, thrombocytopaenia indicate clonal) +PBS
flow cytometry to determine if they are clonal.
Rx:
somewhat dependent on underlying pathology- if stress/ infection then will likely resolve with infection
treat cancer as appropriate.
discuss lymphadenopathy
most often benign, however a pertinant finding.
localised- 75% (50% head and neck)
generalised- 2 distinct non conntected regions.
histology is useful for determining the cause of the lymphadenopathy.
also Use CT and labs to fully investigate.
if readily mobile they are less concerning fro a malignant condition.
Rx:
the underlying condition.
potential causes of:
infectious
malignant
autoimmune
medication
discuss stageing in lymphadenopathy/ lymphoma
1: found in one lymph organ or node
2: 2 or more groups of nodes on the same side of the diaphragm
3: both sides of the diaphragm
4: metastesised to one organ outside the lymphatic system.
Ann arbour classificaiton
causes of splenomegaly + specific cause of concern in the elderly
classified into 4 catorgries- congestive, infiltrative, immune, neoplastic.
liver disease (increaced pressure)
haematologic malignancies
thrombus
splenic sequestration - immune mediated destruction of RBC.
acute / chronic infection
connective tissue disorders.
thinner capsule leading to easier rupture.
presentation of splenomegaly + Ix + RX:
vague abdominal discomfort +/- LUQ pain.
symptoms of the underlying cause are more common.
Ix: CBC + PBS
imaging (CT/US)
Rx: underlying cause
can do splenectomy
main concern is rupture in splenomegaly.
what is multiple myeloma + S+S + Ix
a monoclonal proliferative disorder of plasma cells in the bone marrow
B cells.
presents with bone pain (back- think T spine pain), anaemia. frequent infections seen, significant fatigue.
investigations for:
Serum/urine protein electrophoresis.
bone marrow biopsy
calcium
MRI for bone lesions
FBC for anaemia + calcium.
mamagement of multiple myeloma
consideration of a stem cell tranplantation and initial work up for that. - autologous is prefered
they need induction (dec plasma cells in bone marrow) to occur- this can be done with chaemo or non chamo drugs.
(non- thalidomide, bortezomib, dex)
need a conidiotning regemen- 4-6 doses of drug (mephalan)
autologous usually lasts 2-3 years
allogenic lasts longer but has other side effects (GVhd)
chaemo in non transplant suitable paitents, depending on fitness
define lymphoma
malignant proliferation of lymphocytes accumulating in the lymph nodes.
presents with solid mass lesions/ enlarged lymph nodes.
most are B cell lymphocytes.
discuss hodgkins lymphoma in more detail. (RF, EPI, Clinical features)
hodgkin lymphoma has distinctive Reed-Sternberg cells (2 nuclei- look like an owl)
B cell lymphoma
Risk factors: affeccted sibling, EBV, SLE, post transplant.
Epi:
any age but peaks in 20s, males
clinical features: lymphadenopathy (painless, rubbery, asymetric)- Cervical > axillary > inguinal (upper body mostly)
alcohol-induced pain
splenomegaly + hepatomegaly = jaundice.
B Symptoms.
management of hodgkin lymphoma + prognosis
spread to other tissues is rare
1st line- radiotherapy (complication= lung fibrosis)
2nd- chaemo ABVD (adriamycin, bleomycin, vincristine, decarbazine)
3rd- stem cell transplant.
60-80% 5 year survival but depends on stage and grade.
discuss non-hodgkins lymphoma and the classifications within it
majority of lymphomas, 6th most common malignancy in the UK
most arise from B lymphocites
classification:
Low grade-
follicular- benign for many years
marginal zone lymphoma- related to underlying inflammation (e.g H pylori)
mantle cell- lymphadenopathy with BM involvement.
High Grade:
Diffuse large B-cell- most common
Burkitts- aggressive and associated with the jaw + tumour lysis syndrome( EBV causes).
higher grade responds better to treatment.
management of non-hodgkin lymphoma
If Low grade- active surveilance Rx only if symptomatic with small mollecule inhibitors.
High grade-
Chemo - R-CHOP(Rituximan, cyclophosphamide, vincristine pred)
monoclonal antibodies (rituximab)
radiotherapy
stem cell transplant.
clinical features of lymphomas + investigation pathway
FLAWS
Fever, lethargy, appetite reduction, weight loss, night sweats.
painless lymphadenopathy, hepatosplenomegaly.
Ix: 1st line- FBC + Blood film
2’: lymph node excision- gold standard
3’: CT TAP/ PET
4’: bone marrow aspiration (routine in NHL)
Hodgkin has younger age of onset, contiguous lymph nodes +RS cells
difference between myeloproliferative disorder and myelodysplasic disorders
both have increaced numbers of myeloid lineage cells
in proliferative cells, the quality is maintained
in dysplasic the quality is reduced.
what is polycythaemia
increaced proliferation of red cells. (can also affect platelet and other myeloid cells)
3 types- Vera- JAK2 mutation
relative polycytaemia - as a result of dehydration
COPD- chronic hypoxia.
signs and symptoms of polycythaemia (Vera) + Rx + prognosis.
intense itching- when hot espechilally
splenomegaly
plethora, bleeding
raised Hb + haematocrit
WCC + platelets inc
headache, dizzy, tinnitus
angina, SOB
Rx: venesection
long term aspirin or hydroxycarbamide in higher risk patients.
prognosis:
10-30% progress to myelofibrosis in 10 years
5% to AML @ 10 years
10-16 year mean survival - thrombisis is major risk.
what is essential thrombocythaemia
abnormal proliferation of megakaryocytes
leads to an elevated platelet count. - with normal WBC,RBC
usually an incidental finding on FBC.
thrombosis is a major risk.
JAK2 mutations may support the diagnosis but not diagnostic. other mutations (calreticulin) in 20%
signs and symptoms of thrombocythemia
thrombosis
haemorrhage
erythromelalgia (intense burning pain of extremeties, severe erythema, inc skin temperature)
splenomegaly
Rx:
hydroxycarbamide or anagrelide- megakaryocyte matureation inhibitor
aspirin
Prog:
benign course
may progress to myelofibrosis after 10-20 years.
where do you get fat embolus from
trauma, from the bone marrow- they dont dissolve in the water based blood and clump together.
common blood work changes in infection
most bacterial infections cause a neutrophilia to counter the infection,
viral infections cause lymphocytosis (especially seen in children)
parasitic infections are related to eosinophilia.
reactive thrombocytosis (high plts) to try to control the pathogen and prevent any more entering the body
Sepsis/ prolonged severe infection- neutropenia + thrombocytopenia as they are ‘used up’
haematological changes in cancer
dependant on the specific cancer- e.g bowel- bleeding –> iron deficiency anaemia
some blood cancers cause increased numbers.
chemotherapy can cause anaemia, neutropenia and thrombocytopenia–> risk of neutropaenic sepsis.
Platelet counts can be high with cancers due to iron deficiencies or any bleeding alongside the cancer
