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HAEM/ONC


LOW CELL LINES + THROMBOCYTOPAENIA

PromptCauses of pancytopaenia
ResponseCentral
Marrow failure - aplastic anaemia, myelofibrosis, viral illness,
Marrow infiltration - lukaemia, lymphoma, solid tumours, TB
Deranged marrow - MDS
Drug induced (chemotherapy, sulpha drugs, alcohol)

Peripheral
Hypersplenism
Auto-immune
Severe sepsis
Self-rate:
PromptED investigation of pancytopaenia
ResponseBlood smear/flim looking for blast cells
CXR ? malignancy
US or CT abdo - Possible malignancy/splenomegaly
Coags - ?Coagulopathy
Viral serology - EBV, CMV, HIV
B12/Folate - megaloblastic anaemia
LFT’s - ?liver disease as cause
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PromptCauses of Thrombocytopaenia
ResponseThink Increased destruction, or decreased production
Increased Destruction:
TTP
ITP
Sepsis
HUS
Decreased production:
Haematologic malignant
Bone marrow suppression secondary to virus or drugs
Cirrhosis/alcohol
Nutritional - B12, folate etc.
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PromptPlatelet goals for procedure:
ResponseICH >100
Lumbar puncture >50
CVC aim >20
Asymptomatic aim >10
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Prompt
ResponsePatechiae and purpura on both legs
Bruising to upper thigh

Further investigations
Coags - Bleeding disorder/DIC
EUC - AKI in microangiopathic haemolytic anaemia (MAHA) or TTP
CT head if platelets low and trauma
LFT - DIC and haematologic malignancy
Crossmatch - ? need for transfusion

Causes:
Blood Clotting problems - Platelets/Clotting
Vessels leaking problems
  • Vasculitis (immune complex mediated - IgA, SLE , PosiImmune mediated - MPA, GPA)
  • Vasculopathy (trauma, amyloid, scurvy, Ehlers Danlos)
Self-rate:
PromptTTP
(See paeds also)
ResponseFAT RN
Fever, micoangiopathic haemolytic anaemia, Thrombocytopaenia, Renal impairment, Neurologic - CNS changes (Headache, altered mental status, coma)
(note same mnemonic for HUS. HUS = more renal, TTP = more neuro. Both are clinically very similar, but different aetiology)

Fragmented red cells/schistocytes on film
Treatment - Plasma exchange, steroids. Avoid platelet transfusion (unless uncontrolled bleeding)

Thrombotic Thrombocytopaenic Purpura (TTP) a rare disorder of coagulation caused by deficiency or inhibition of ADAMSTS13, a protein that is responsible for breaking down von Willebrand factor. This results in haemolytic anaemia, thrombocytopaenia, and fluctuating neurological abnormalities - It is treated by plasmapheresis
- Oxford medical dictionary
Self-rate:
PromptITP
ResponseNo evidence of haemolysis.
Platelet bound antibodies definitive diagnosis
Treatment - Prednisone (vs. pulsed methylpred), Immunoglobulin, platelet transfusion

Immune thrombocytopaenic Purpura: (Prev. Idiopathic thrombocytopaenic purpura) is an autoimmunie disease in which platelets are destroyed, leading to spontaneous bruising. Acute ITP is a relatively mild disease of children who usually recover without treatment. A chronic form of the disease, typically affecting adults, is more serious, requiring treatment with corticosteroids or, if there is no response, with splenectomy. If both fail, immunosuppressant drugs may be effective. Platelet concentrates are used for life threatening bleeding - Oxford medical dictionary
Self-rate:
PromptHUS vs TTP vs ITP
ResponseHSP (now IgA vasculitis) = normal plt/coags - ie: inflamed vessels leak >> patechiae
ITP - Low plt, other cell lines OK. probably mild in kids
TTP - Haemolytic anaemia, thrombocytopaenia, renal failure, looks sick.
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PromptSepsis
ResponseCoags looking for DIC
Mgmt - source control/IVABx, DIC management
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PromptMalignancy
ResponseBlood flim looking for signs of malignancy then bone marrow
Mgmt - Oncology/haematology referral
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PromptHUS
See paeds also
ResponseFragmented red cells/schistocytes
Renal function for AKI
Management - fluid and electrolyte management
Often preceded by gastro (E. Coli 0157:H7)
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PromptBone marrow suppression
ResponseBone marrow biopsy
Diagnoise and treat cause (eg: cease offending drug/chemothrapy)
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