Haematology Flashcards
Myeloma prognostic markers
B2 microglobulin and Albumin
high and low respectively is bad prog
febrile and jaundiced 7 days after blood transfusion, management
Supportive mx unless symp anaemia
delayed transfusion reaction, typically 7-10 days post-tran
2ndary to sensitised from previous transfusions/preg; alloantibodies against red cell antigens
Patient w flu-like symptoms headache, GI symps, haemorrhagic fever
Been in bat cave in Uganda
Marbug virus
Auer rods seen it…
pathognomonic of Acute promyelocytic leukaemia M3
Assx w t(15;17)
M3 subtype of AML
Fever/chills after blood transfusion
As long as no hypotension, dyspnoea wheezing Angioedema, Abdo pain etc
Slow/stop transfusion, paracetamol and monitor
Likely non haemolytic febrile reaction
Blood film: schistocytes
Low platelets
High APTT and PT
DIC
Schistocytesdue to microangiopathic haemolytic anaemia
Commonest cause of chronic DIC in elderly
Prostate cancer
acquired factor VIII deficiency Vs Von willebrands
Both cause long APTT but in Vwb APTT corrects with addition of plasma
Most common cause of hyperviscocity syndrome
Waldenström’s macroglobulinaemia (most common cause).
Presents with neuro symps like headaches, visual disturbances, papilloedema, hypertension, bleeding.,.
Treat with plasmapharesis to rapidly reduce viscosity and reduced thromboembolic events
Patient with Waldenström’s macroglobulinaemia presents with headaches, visual disturbances, papilloedema, hypertension, bleeding.,.
Hyperviscocity syndrome
Treat with plasmapheresis
Management of heparin induced thrombocytopenia
Switch to direct thrombin inhibitors like Argatroban or danaparoid
remember: low platelets but is actually a prothrombotic condition
and HIT antibodies have a high false positive rate
Can use Warkentin probability scale
Treatment of atypical HUS
Eculizumab (a C5 inhibitor)
fever, anaemia, thrombocytopenia, renal failure and confusion post partum.
Fragmented red cells on blood film
Thrombotic thrombocytopenic purpura
overlaps with HUS, but HUS is classically after bloody diarrhoea (EColi0157:H7) and more severe AKI
Management of Granulomatosis with polyangiitis (Wegener’s granulomatosis)
Cyclophosphamide and steroids (90% response)
Complications of plasma exchange
Hypocalcaemia (due to citrate as anticoag)
Metabolic all
Coagulation depletion so bleeding risk
Reed-Sternberg cells
hodgkin’s
which hodgkin’s has worst prognosis?
Lymphocyte depleted (also rarest)
which hodgkin’s has best prognosis?
Lymphocyte predominant
alcohol-induced pain?
hodgkin’s lymphoma
raised reticulocyte and LDH
Haemolysis
high reticulocyte count (medically known as reticulocytosis) can be found after blood loss due to injury, ulcers, or surgery
LDH is found intracellularly, so indicates damage…
raised red cell count, platelets and splenomegaly
polycythaemia vera
Would usually have raised Hb but could be normal if co-existing IDA
The most common cause of an isolated lymphocytosis in an adult
Chronic lymphocytic leukaemia (CLL)
Tx if constitutional symptoms
Anti-CD20 monoclonal antibody (e.g. obinutuzumab), chlorambucil or bendamustine
red cell fragmentation
heart valves
TTP
DIC
HUS
thrombotic thrombocytopenic purpura vs haemolytic uraemic syndrome
Overlap, but HUS is classically after bloody diarrhoea (EColi0157:H7) and more severe AKI
both can have fragmentation of red cells on blood film
DIC would have low platelets, prolonged APTT, PT
ADAMTS13 deficiency
TTP
ADAMTS13 Deficiency (a metalloprotease enzyme) which cleaves large multimers of von Willebrand’s factor
What is the deficiency in TTP
ADAMTS13
Which breakdowns large multimers of von Willebrand’s factor
alternative description for Auer rods
needle-like elongated cytoplasmic inclusions
pathognomonic of acute myeloid leukaemia
who do you screen for thrombophilia?
younger pt w spontaneous clotting events
don’t test during acute event though or on anticoag
commonest thrombophilia
Factor V Leiden (heterozygous)
5%
x4risk
most severe thrombophilia
Antithrombin III deficiency
rare 0.02%
x10-20 risk
ITP vs TTP
ITP: autoimmune destruction of platelets and is managed with immunosuppression - the first line is usually prednisone, IgGlob
TTP platelets clump within vessels due to deficiency of ADAMTS13 Fluctuating neuro, AKI, Raised LDH Medical emergency Plasma exchange.
when do you treat Chronic lymphocytic leukaemia (CLL)?
B symps
worsening of anaemia and/or thrombocytopenia
>10 cm or progressive lymphadenopathy
>6 cm or progressive splenomegaly
lymphocytosis: > 50% increase over 2 months or lymphocyte doubling time < 6 months
autoimmune cytopaenias e.g. ITP
commonest cause of amyloidosis in uk
plasma cell disorders (e.g. myeloma) are the commonest cause of amyloidosis in the UK and lead to primary (AL) amyloidosis
old person w anaemia, neutropaenia, thrombocytopenia
myelodysplasia
25 yr old presents acutely unwell w recent weight loss, nosebleed, oozing from his cannula sites and non-blanching purpura over his arms and legs. INR 8.5 PT 89 s (9-12) APTT ratio 1.7 (0.8-1.2) Platelets 43
DIC, in acute promyelocytic leukaemia (APML presents younger than other AML)
Tx Tretinoin AKA all-trans retinoic acid
HSP doesn’t have pancytopenia/bleeding
Aplastic anaemia wouldn’t have weight loss
how do you assess for ?heparin induced thrombocytopenia
HIT antibodies have a high false positive rate
Can use Warkentin probability scale
4Ts Score
- Thrombocytopenia (30-50, >50)
- timing 5-10d, or <1d if 100d since exposure
- Thrombosis (thrombosis or skin necrosis)
- other cause for thrombocytopenia
what is Berger disease aka
IgA nephropathy
what is IgA nephropathy aka
Berger disease
bloody diarrhoea then AKI and thrombocytopenia… what type of haemolytic anaemia?
microangiopathic haemolytic anaemia (MAHA) in the context of HUS-TTP
immediate therapy for acute promyelocytic leukaemia
Tretinoin PO for 3/12
AKA all-trans retinoic acid (ATRA)
If diagnosed, one of the most treatable leukaemias, however, 1/3 present w catastrophic haemorrhage
Prognosis with Philadelphia translocation t(9;22)
GOOD in CML (most commonly found)
BAD in AML + ALL
