haem Flashcards by Amy Hollis

investigations for sickle cell?

initial= FBC
-normal/ low MCV
-low HB

gold standard= haemoglobin electrophoresis
-Hbs
-no/ low HbA

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treatment- sickle cell?

Acute crisis:
-analgesia
-IV fluids, rest, O2
-chest/ neruo symptoms consider exchange transfusion

prophylaxis:
-Hydroxyurea
or
-regular transfusions

prophylactic penicillin + pneumococcal vaccine if hyposplenism

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genetic mutation- sickle cell?

autosomal recessive
-point mutation in codon 6 of beta globin gene
-substitutes glutamine to valine producing bs

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investigations haemochromatosis?

transferrin saturation (raised >50%)= most useful
-genetic testing for HFE for 1st degree family

other bloods:
-serum ferritin increased
-Low TIBC

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management of hereditary haemochromatosis?

1st= weekly venesection
-monitor transferrin saturation <50% + ferritin <50

2nd= deferrioxamine (iron chelating drug)

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management of B12 + folate deificiency?

treat B before F

1st= hydroxocobalamin IM (B12) 3x a week for 2 weeks then once every 3 months

2nd= folic acid

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investigation perniscious anaemia?

intrinsic factor antiobodies (more specific than gastric parietal)

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blood results VWF deficiency

normal platelet
normal PT
APTT may be prolonged or normal

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inheritance VWF deficiency

autosomal dominant

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management VWF deficiency?

day to day most dont require treatment

moderate VWF= desmopressin

may also use: factor 8 infusion or VWF infusion

mild bleeding= tranexamic acid

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ITP blood results

isolated thrombocytopenia

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ITP management children

Self limiting (6 months)
-avoid contact sport

platelets <10
-prednisolone
-IV immunoglobins

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ITP management adults

1st= prednisolone (oral)
other= pooled IVIG (quick + good if active bleeding or urgent procedure required)

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bloods of haemophilia?

isolated prolonged APTT

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genetics of haemophilia + clotting factors affected

X linked recessive (usually boys affected)

haemophilia A= factor 8
haemophilia B= factor 9

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management of thrombotic thrombocytopenic purpura?

-plasma exchange
-steroids
-rituximab

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what causes thrombotic thrombocytopenic purpura

deficiency in ADAMST13 protein which leads to overactive VWF
-(causing more platelet adhesion leading to microangiopathy)

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patient starts heparin, 5-10 days later develops blood clots + low platelets

-what do you do

heparin induced thrombocytopenia

-test for anti PF4/ heparin antibodies

stop heparin + talk to specialist about changing to another anticoag

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haemophilia management?

infuse the missing clotting factors

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most common thrombophilia UK

Factor V leiden (Factor V becomes resistant to protein C)

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management of factor V leiden

-advice on risk (avoid COC, smoking)

-short term prophylaxis to prevent thrombotic events during high risk periods

-recurrent thrombotic events= long term anticoagulant

antiphospholipid syndrome management

primary prophylaxis or pregnant:
aspirin + LMWH

has a thromboembolic event:
lifelong warfarin (INR 2-3)

recurrent thromboembolic events on warfarin:
increase INR 3-4

investigations antiphospholipid

increased APTT
thrombocyotpenia

anticardiolipin
anti beta 2 glycoprotein

women comes in 7-14 days post chemo

neutrophil count <0.5
temp >38
crackles in chest

managment?

immediately start= piperacilin + tazobacam (tazocin)

still unwell after 48 hours= meropanem +/- vanc

SEPSIS 6
Blood cultures
UO
Fluids
Antibiotics
Lactate
Oxygen

most common organism causing neutropenic sepsis?

coagulase-negative, Gram-positive bacteria Staphylococcus epidermidis (perhaps due to indwelling lines)

A 45-year-old female presents to the emergency department complaining of pain in her right leg. She has recently returned from an international business trip and reports the sudden onset of this pain earlier in the day. On examination, the right calf is swollen and tender on palpation. There is also notable pitting oedema that extends to the mid-shin. The right calf is measured as 4 cm larger than the left. The d-dimer is found to be elevated at 1043 ng/ml (<500 ng/ml). Given the likely diagnosis, the patient is started on anticoagulation whilst awaiting an urgent ultrasound, as it cannot be performed within four hours. The result of this scan, however, is negative. What is the most appropriate action?

stop anticoagulation and arrange repeat US in 1 week

medications that can cause aplastic anaemia?

phenytoin chloramphenicol

investigation for suspected autoimmune haemolytic anaemia?

Coombs test (will be +ve) other bloods: -low Hb -reticulocytosis -high bilirubin -raised LDH blood film= spherocytes + reticulocytes

cause of warm autoimmune haemolytic anaemia?

idiopathic AI (SLE) neoplasia (lymphoma + CLL) drugs e.g. methyldopa IgG mediated extravascular haemolysis

treatment warm autoimmune haemolytic anaemia?

Treat underlying cause 1st line= steroids +/- rituximab

cause of cold autoimmune haemolytic anaemia?

neoplasia e.g. lymphoma infections e.g. EBV or mycoplasma IgM mediated intravacular

what is given to patients with polycythaemia vera?

1st line= venesect until haematocrit <0.45 aspirin (reduce risk of clots) cytotoxic chemo (hydroxycarbamide)

night sweats, weight loss, fever lymphadenopathy painful after alcohol -what will be found on histology?

Hodgkins lymphoma -Reed steingberg cells

reversal agent for dabigatran?

Idarucizumab -dabigatran is a DOAC that works by directly inhibiting thrombin

typical bloods seen in DIC

↓ platelets ↓ fibrinogen ↑ PT & APTT ↑ fibrinogen degradation products schistocytes due to microangiopathic haemolytic anaemia

what types of hodgkins lymphoma have best + worst prognosis

best= lymphocyte dominant worst= lymphocyte depleted

how is hodgkins lymphoma diagnosed + staged?

EXCISIONAL biopsy (remove the whole area) + PET/CT for stageing use ANN ARBOR stageing I: single lymph node II: 2 or more lymph nodes/regions on the same side of the diaphragm III: nodes on both sides of the diaphragm IV: spread beyond lymph nodes

A 67-year-old man comes in complaining of lethargy, weight loss and night sweats in the last 3 months. As part of his investigation, the man has a bone marrow aspiration which shows an increase in granulocytes at different stages of maturation under the microscope. What is the first-line treatment for this condition?

he has CML imatinib (tyrosine kinase inhibitor)

what is a raised ESR + oesteoperosis until proven otherwise?

multiple myeloma

what would bone marrow aspirate, peripheral blood film, protein electrophoresis of urine, serum electrophoresis + Xray of multiple myeloma show

bone marrow aspiration= plasma cells peripheral blood film= rouleux formation urine electrophoresis= bence jones proteins serum electrophoresis= raised concentrations of monoclonal IgA/ IgG Xray= rain drop skull

imageing recommended for myeloma

whole body MRI

anaemia of chronic disease bloods - normal MCV

high ferritin low serum iron low transferrin saturation Low TIBC

anaemia of iron deficiency bloods

Low ferritin Low serum iron Low MCV low transferrin saturation high TIBC

what is ritchers phemenon?

Occurs in CLL!! Ritcher's transformation occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin's lymphoma. Patients often become unwell very suddenly. Ritcher's transformation is indicated by one of the following symptoms: -lymph node swelling -fever without infection -weight loss -night sweats -nausea -abdominal pain

Fever, abdominal pain, hypotension during a blood transfusion management?

probs acute haemolytic reaction Stop transfusion Confirm diagnosis check the identity of patient/name on blood product send blood for direct Coombs test, repeat typing and cross-matching Supportive care fluid resuscitation

where is B12, folate and iron absorbed

B12= ileum folate= duodenum + jejunum iron= jejunum (needs Vit C + acidic environment)

what is feltys syndrome

RA splenomegaly Low WCC

how do the most common metastatic cancers to bone present on spine Xray?

PB KTL Prostate - osteoblastic (whiter) Breast- mixed Kidney- lytic (darker) Thyroid- lytic (darker) Lung- lytic (darker)

who is at higher risk of AML?

>65 years -people with already known myelodysplastic syndrome

investigations ALL?

bone marrow biopsy -confirms diagnosis + immunophenotyping FBC: -anaemia -thrombocytopenia -low/ normal/ high WBCC

investigation + management EBV?

heretophii antibody test (Mono spot) rest, analgesia, avoid alcohol -avoid contact sport for 4 weeks