hematology incorrects Flashcards
75 YO woman recieveing 3rd unit of blood. sats 90%, bibasal inspiratory crackles, occasional wheeze
treated with oxygen
next step in management?
IV furosemide!!!
patient is fluid overloaded
A 78 year old woman has a chest infection that is slow to clear despite several
courses of broad spectrum oral antibiotics. Investigations: Haemoglobin 126
g/L (115 – 150) White cell count 38 x 109
/L (3.8–10.0) Lymphocytes 32 x
109
/L (1.1–3.3) Platelets 124 x 109
/L (150 – 400) A large cervical
lymph node is biopsied and shows a sheet of mainly small lymphoid cells with
5% larger cells.
Which is the most likely diagnosis?
chronic lymphocytic luekemia - diagnosed by high lymphocyte count in peripheral blood count
NOT non hodgkins lymphoma
what pathogen commonly causes neutropenic sepsis in cancer patients?
Coagulase-negative, Gram-positive bacteria such as Staphylococcus epidermidis
usually due to indwelling lines
A 28-year-old lady comes into your clinic. She expresses concern that her periods are getting heavier and that her nosebleeds are becoming more frequent. On examination, she is well and afebrile and has a non-blanching rash on the flexor surface of the arms. Past medical history includes diagnosis of asthma and lupus. A blood test reveals the following:
ITP can be clinically diagnosed with an isolated thrombocytopenia.
Many patients may present with anaemia and purpura due to the blood loss and low platelets.
APTT is typically increased in both haemophilia A, VWD and vitamin K. TTP is a serious condition with anaemia, fever, purpura and cerebral dysfunction.
A 25-year-old man with a history of sickle cell disease presents to the emergency department with severe chest pain, fever, and shortness of breath. His oxygen saturation is 88% on room air, and a chest X-ray shows new pulmonary infiltrates.
After initiating oxygen therapy and analgesia, what is the most appropriate next step in managing this patient?
exchange transfusion!!!
indications include: acute vaso-occlusive crisis (stroke, acute chest syndrome, multiorgan failure, splenic sequestration crisis.reduces number of HBs
blood transfusion given for symptomatic anemia and antibiiotics as infection can trigger ACS but an exchange transfusion would be the most appropriate immediate next step
name a recreational drug that can cause methemylglobulinemia
nitrates
what is the antibiotic of choice for neutropenic sepsis?
piperacillin with tazobactam - tazocin
in immunocompormised patients, what blood product poses the greatest risk?
platelets
highest risk of bacterial contamination
person presents with lymphocytosis and neutropenia following treatment for suspected recurrent tonsillitis.
most likely cause?
glandular fever also known as infectious mononucleosis) caused by the Ebstein-Barr virus. An increase in the number of circulating activated T and B lymphocytes seen in the virus causes lymphocytosis. Neutropenia can also be caused by the virus, however, the aetiology is not fully understood.
lymphoma would cause high lymphocytes but not neutropenia
a tonsillar abscess would cause high neutrophils
ITP
Investigations?
first line treatment?
antiplatelet autoantibodies (usually IgG)
bone marrow aspiration shows megakaryocytes in the marrow. This should be carried out prior to the commencement of steroids in order to rule out leukaemia
treatment = oral prednisolone!!
splenectomy if platelets < 30 after 3 months of steroid therapy
what is the platelet transfusion threshold?
a platelet count of <30 x 10 9 with clinically significant bleeding
e.g. haematemesis, melaena, prolonged epistaxis)
< 100 x 10 9) for patients with severe bleeding, or bleeding at critical sites, such as the CNS.
no bleeding = threshold of 10 x 109
why does SLE cause neutropenia?
autoimmune destruction of neutrophils
distingusih between the crises in sickle cell anemia and how they present
sequestration crises - worsening of anemia or SOB due to pooling of rbcs - INCREASED reticulocytes
aplastic crises - REDUCED reticulocyte coubt
hemolytic crises - rare - pain and jaundice
polycythemia vera may progress to what malignancies?
acute myeloid leukemia!!!! or myelofibrosis
A 55-year-old woman presents to the emergency department with new-onset dysuria. She denies any cough, shortness of breath, nausea or vomiting, or changes in bowel habits. She has a medical history of breast cancer currently being treated with doxorubicin and cyclophosphamide.
On examination, her temperature is 38.1ºC,
What is the best next step in her management?
immediate IV tazocin
fever + chemo = neutoropenic sepsis risk
uti may well be source but must treat neutropenic sepsis first
patient did not report blood in urine
what are the typical lab values in DIC caused by HELLP
s low platelets, low fibrinogen, prolonged PT, prolonged activated partial thromboplastin time (APTT), and raised fibrinogen degradation products.
what must the platelets be above to proceed with a surgery or an invasive procedure?
50 x10^9
if not give platelet transfusion first
hodgkins lymhopma, swelling in neck
first line investigation?
lymph node biopsy
ct scan of chest and abdomen and PET scan are only used later
prophylaxis fpr tumour lysis syndrome?
allopurinol!! or rasburicase
patient who is on apixaban has hemorhagic stroke
what is the most appropriate medication to prescribe for this patient?
Adexanet alfa = reverses Apixaban and rivaroxaban
digabatran is reversed using idarucizumab!
hodgkins lymphoma can present with normal lymphocyte count!
you may just notice anemia and esoinophilia + swelling in neck
differentiate the finidings in beta thalessemia trait vs beta thalessemia major
in beta thalessemia trait, hemoglobin rarely drops below 90 even though symptomatic anemia present
in beta thalessemia major, profound anemia, often less than 60
what type of anemia does coeliac disease cause?
microcytic anemia
iron deficiency due to reduced absorption
Venous thromoboembolism - length of anticoagulation
provoked (e.g. recent surgery): 3 months
unprovoked: 6 months
rivaroxaban, digabatran are options. if allergic give warfarin
alteplase is a thrombolytic used in emergency situations not long term
taco vs trali findings
febrile hemolytic reaction vs non febrile acute hemolytic reaction
anaphylactic reaction findings
Transfusion Associated Circularory Overload: Hypertension!!, raised jugular venous pulse, afebrile, S3 present, pulmonary edema may occur
Transfusion Related Acute Lung Injury: Hypotension!!!, pyrexia!!, normal/unchanged JVP, pulmonary infiltrates/inspiratory crepitations!!! due to non cardiogenic pulmonary edema
hypotension and abdominal pain help distinguish acute hemolytic reaction (the diagnosis) from febrile no-hemolytic reactio
expiratory wheeze, vs pulmonary crepitations in TRALI. anaphylactic reaction also has quicker onset eg in minutes and also causes hypotension like trali
A 67-year-old man presents to his GP with bleeding after brushing his teeth. Five years ago, he completed his final radiotherapy course of 55Gy (gray) units in 20 fractions for bladder cancer.
blood tests show pancytopenia?
most likely diagnosis?
pancytopenia 5 years post chemo/radiotherapy -> myelodysplastic syndrome
(can present with fatigue, shortness of breath, weakness, pallor, splenomegaly, easy bruising/bleeding, petechiae and sometimes bone pain. T)
contrast to myelofibrosis which would also present with b symptoms and hepatospleenomegaly
A 28-year-old female is attends the gynaecology unit for a D+C following an incomplete miscarriage. She has previously had recurrent pulmonary embolic events. After the procedure she is persistently bleeding. Her APTT is 52 (increased).
most likely diagnosis?
anti-phospholipid syndrome
A 70-year-old coal miner presents with 3 weeks of haematuria and bruising. He is normally fit and well. He is on no medications. His results reveal:
Hb 9.0
WCC 11
Pl 255
PT 16 (normal)
APTT 58 (increased)
Thrombin time 20 (normal).
acquired hemophilia
factor 8 acquired disorder
The elderly, pregnancy, malignancy and autoimmune conditions are associated with acquired haemophilia. Prolonged APTT is key to the diagnosis. Management involves steroids.
what comprises cyroprecipitate?
factor VIII!!!, fibrinogen, von Willebrand factor and factor XIII
how does itp present
treatment?
petiechia or episataxis + low platelets
oral pred !! - ITP is a destructive pathology of platelets so platelet transfusions have little to no effect
sometimes presents with massive bleeding eg intracranial
name a cancer that is more likely to cause tumour lysis syndrome
burkitts lymphoma
cancers with high wcc essentially
target cells and what else can be seen on blood film in coeliac disease?
howell jolly bodies
due to hyposplenism
name conditions with low platelet count where platelet traqnsfusions are contraindicated
Chronic bone marrow failure
Autoimmune thrombocytopenia - oral prednisolone to treat
Heparin-induced thrombocytopenia, or
Thrombotic thrombocytopenic purpura - this is treated with corticosteroids
which sikle cell drug is used to decrease the incidence of complications/crises?
hydroxyurea/hyroxycarbamide
when would you do a ddimer vs ultrasound for DVT
wells score
wells score 2 or more = leg ultrasound
wells score <2= d dimer
digabatran mechanism of action?
direct thrombin inhibitor
Myelodysplastric syndrome can undergo transformation into what condition?
acute Meyloid leukemia
m=m
in cases of major hemorrhage, how is tranexamic acid administered?
IV bolus followed by SLOW infusion
name an antipsychotic associated with increased risk of VTE
olanzapine
VWD is the most common inherited bleeding disorder
what should be prescribed to patients with polycythemia vera and why?
aspirin
to decrease risk of thrombotic events
3rd generation cocps have higher risk of vte than 2nd generation cocps
what is the key triad for management of sickle cell crisis
opiates + oxygen + IV fluids
what is the first line treatment for autoimmune hemolytic anemia?
corticosteroids - to reduce the amount of circulating auto-antibodies
+/- rituximab
how does pernicious anemia present? what do you check as first line investigation
b12 deficiency = macrocytic anemia
intrinsic factor antibodies
if investigating a suspected DVT, and either the D-dimer or scan cannot be done within 4 hours, next step in investigation?
commence on DOAC whilst waiting
A 21-year-old woman presents to the emergency department with her mother with a 2-day history of severe generalised abdominal pain and arm weakness. She has been feeling very low in mood recently and has been struggling to sleep, and her mother is concerned that she has seemed confused today. Her mother reports having had similar episodes to this herself in the past. On examination, her hands and eyes appear normal. Her abdomen is soft and there are no palpable masses. Upper limb power is 3/5 and upper limb reflexes are absent. Sensation is intact. Lower limbs are normal.
What is the most likely diagnosis?
acute intermittent porphyria!!
-> triad of abdominal, neurological and psychiatric symptoms
what is the first line treatment for VTEs (PEs or DVTs)
(DOACs) - specifically APIXABAN or RIVAROXABAN, including as interim anticoagulants before a definite diagnosis is made
lmwh no longer first choice!!
recommended treatment for post-thrombotic syndrome?
graduated compression stockings
59-year-old baker presents to his general practitioner complaining of tiredness that has been getting worse over the past 6 months. On examination, he is pale without jaundice, and there is a non-tender mass in the left upper quadrant. He has a past medical history of tuberculosis, for which he has been on treatment for 20 years. A blood test is performed which shows the following:
hypochromic mycrocytic anemia
high ferritin and transferrin saturation
basophilluc stippling of rbcs
most likley diagnosis?
sideroblastic anemia
anemia + hepatospleenomegaly
congenital or acquired (anti-tb medication!!, lead, alcohol, myelodysplasia)
a differential will be lead posioning which also causes microcytic anemia and basphilic stippling. but patient has no risk factors for lead poisioning eg battery construction, old housing, heavy construction). lead poisoning causes abdominal pain, neuropathy and constipation. (no psychiatric symptoms like AIP) (anemia is not a feature of AIP)
anemia of chronic disease would cause a normocytic anemia, is not associated with organomegaly and would cause low transferrin saturation.
most likely lymph nodes site of metastasis for ovarian cancer?
para-aortic lymph nodes
A 35-year-old man has presented to his GP complaining of feeling increasingly tired and getting short of breath after minimal activity. He has Crohn’s disease which resulted in him needing an ileocaecal resection 1 year ago due to an uncontrollable flare up. However currently his Crohn’s disease is well controlled. On examination his skin and conjunctiva appear pale. His tongue also appears beefy-red and is sore. He is tachycardic with a heart rate of 110 bpm.
vitamin B12 deficiency due to resection of ileum
glossitis seen in B12 deficiency
most common cause of crises in sickle cell?
dehydration!!!
or infection
uriticaria during blood transfusion, no blood pressure or breathing compromise. how do you manage?
minor allergic reactions may be managed by temporarily!!! stopping the transfusion and giving an antihistamine
62 YO man presenting with iron deficiency anemia. first step in management?
refer for colonoscopy via 2ww!!!
for all patients over 60 presenting this way
transfusion threshold for patients with ACS/mi?
hemoglobin 80g/l
in patients with both vitamin B12 and folate deficiencies, what is the first line in management?
B12 injections!!
vitamin B12 deficiency must be treated first to avoid subacute combined degeneration of spinal cord
what must be present to confirm a diagnosis of tumour lysis syndrome?
increased serum creatinine, a cardiac arrhythmia or a seizure
unlike beta thalessema major, trait is usually asymptomatic and anemia is mild
how often should Sickle cell patients should receive the pneumococcal polysaccharide vaccine?
every 5 years
proximall!!!! leg ultrasound not distal leg ultraosund for DVT
incontrast to beta thalessemia, reticulocytes are low in parvovirus b19 and aplastic anemia
what anti-diabetic drug is contra-indicated in G6PD?
sulfurnylureas eg GLIPIZIDE!!
A 21-year-old man comes for review. He recently had an abdominal ultrasound for episodic right upper quadrant pain which demonstrated gallstones. A full blood count shows normocytic anemia
The patient also mentions that his father had a splenectomy at the age of 30 years.
Which one of the following tests is most likely to be diagnostic?
EMA binding test
hereditary spherocytosis
TTP presentation
name some drugs that cause aplastic anemia
cytotoxics, chloramphenicol, sulphonamides, phenytoin!!!
Alcohol toxicity can cause a macrocytic anemia but hyper-segmented cells would not be seen on the blood film.
A 68-year-old man who takes warfarin for atrial fibrillation is taken to the emergency department after being involved in a road traffic accident. His GCS is reduced and a CT head shows an intracranial haemorrhage. Bloods on admission show the following:
INR 3.1
In addition to vitamin K, which one of the following blood products should be given?
prothrombin complex concentrate - used to reverse effects of warfarin in patients with life threatening bleed
has a faster onset than FFP which is used in liver disease and dic
A 68-year-old man presents with sudden onset hemiparesis affecting the left face, arm and leg. On examination, you note left-sided hemiparesis and a left homonymous hemianopia. You also note lymphadenopathy and splenomegaly. Further investigations reveal an IgM paraprotein of 40 g/L and a skeletal survey shows no bone lesions. A blood test demonstrates normal calcium and creatinine
most underlying cause of the stroke?
waldenstoms macroglobulinemia
A 76-year-old man presents to his GP with a lump in his groin and testicle. Upon further questioning, he reports significant weight loss, lethargy, and night sweats over the past 3 months.
Upon examination, the doctor notes painless lymphadenopathy of the left inguinal and right cervical lymph nodes that feel rubbery in texture. Hepatomegaly is also noted and a mass is felt on palpation of the right testicle.
anemia, normal wbc count. what is most likely diagnosis? First line investigation?
non hodgkins lymphoma!! (more common than hodgkins)
lymph node biopsy
CML in contrast would present with spleenomegaly
excessive bleeding after tooth extraction. prolonged APTT only. which clotting factor is most likely to be deficient?
factor 8 - patient most likely has hemophilia a
how to confirm a diagnosis of G6PD deficiency?
G6PD enzyme assay at time of presentation and at 3 months
In a man who is three days post prostate biopsy, presenting with confusion and rigors, an neutropenia, what is an important differential?
urosepsis due to biopsy
what should be prescribed between units of packed rbcs?
furosemide
as transfusions can result in fluid overload
what is the red blood cells transfusion threshold?
70 g/l hb in patients without ACS
80 g/l in patients with ACS
A 69-year-old woman is in hospital receiving treatment for suspected Mycoplasma pneumonia. In addition to a cough, chest pain and ongoing fatigue the woman also develops palpitations, pale skin and darker-coloured urine.
hemolytic anemia evidenced by bloods. what test is useful in identifying the suspected complication?
coombs test. patient is experiencing autoimmune hemolytic anemia
hereditary spheroytosis and g6pd have no linke to mycoplasma
anemia of chronic disease lab values?
ferritin is high, everything else low
cancer related DVT management?
DOAC for SIX months
A 36-year-old woman is brought to the emergency department with severe abdominal pain, vomiting and confusion, which began 2 hours ago. She is agitated and describes auditory hallucinations. The patient had two seizures before she arrived at the hospital.
She has a past medical history of depression and recurrent UTI, and she has recently been prescribed sertraline and a course of nitrofurantoin.
She has a heart rate of 160bpm, blood pressure of 180/110mmHg and is hypo-reflexive.
A urine sample is sent to the lab for analysis and noted to have turned a deep red on standing.
What is the most likely diagnosis?
Acute intermittent porphyria - classically urine turns deep red on standing!!
TP typically presents a pentad of neurological symptoms, renal dysfunction, fever, haemolytic anaemia and thrombocytopenia. These patients are likely to be jaundiced with evidence of bleeding into the skin/mucous membranes and would be unlikely to present with psychiatric manifestations, abdominal pain/vomiting or red urine.
in an acute hemolytic transfusion reaction, what type of antibody binds?
IgM
Mechanical aortic valve replacement 63yo. And coronary revascularisation 3 days ago. Takes aspirin long term. Treated with deltaparin sodium.
Most appropriate long term management?
Warfarin sodium!!!
Patients with mechanical heart valves require aspirin and warfarin. Lmwh used short but not long term