Haematology Flashcards
patient had a high fever, high fever (>40), shock, rigors
Bacterial contamination of the unit
one history had a patient who had recently been involved in some kind of trauma and had been transfused with O (universal donor) negative blood. He lost consciousness or something a few minutes after the transfusion and the answer was
haemorrhage with a splenic haemorrhage after a stab wound rather than ABO incompatibility (because O negative blood).
Woman with O (universal donor) rh+ (can receive both) given A rh- (can only recive rh(-), what happens
Immediate haemolytic reaction, e.g. ABO mismatch, massive intravascular haemolysis
Lady who received a blood transfusion and platelets and went home and developed a rash all over and “some shortness of breath”
(mild allergic reaction – if severe (wheeze))
Elderly patient after a road traffic collision who required a massive transfusion - What is the most serious complication that could happen, 6 hours post, leading to macrophage infilitration into the lungs (non-cardiopgenic pulmonary oedema)
TRALI –trial of dononr antibodies leaving the TRAIL of antibodies into the recipient
Woman who received a transfusion before her hysterectomy who presented with bleeding gums and bruising rash on her shins on discharge
Post-transfusion purpura (PTP)
the patient makes an alloantibody in response to platelet antigens in the transfused blood that for a period of time causes destruction of autologous antigen negative platelets
. Post transfusion purpura: HLP1A
> 24 hours with extra-vascular haemolysis (jaundice, haemoglobinuria)
Delayed Haemolysis Transfusion. Occur if patients had previous transfusions or pregnacies, antibodies agains
red cell antigens
maculopapular rash, diarrhoea
GVHD
severe anaphylaxis, laryngeal edema, wheeze, raised pulse
IgA deficiency
dry cough, fever, dyspnoea low JVP, anti leukocyte antigens, acute SOB, low sats
TRALI
same as TRALI but raised BP
TACO
everything else excluded but high fever and hypotension-
bacterial infection of the unit
mild symptoms (fever 2-h ago), raised pulse
Febrile non haemolytic:
thalassemia patient who looks tanned and has diabetes
Transfusion haemosiderosis
Already transfused once before –> sensitized
anaphylaxis due to IgA def (Immediate death: wrong blood, anaphylaxis (often goes with IgA) if managed poorly
they have lots of transfusions so might have uncommon antibodies check antibodies in depth
Sickle cell
Woman with anti D before C section - CMV neg
cross match
Man with AAA repair (elective)
cross match
Woman in emergency
O neg
Immunocompromised
irradiated
Previous severe allergic reaction
washed red cell and platelets
A plastic anemia needs monthly transfusion irradiated and filtered
platelets if <10,000 and if bleeding, depleted
non-cancerous growth in lymph nodes
HHV 8 Castleman’s disease
causes post-transplant lymphoproliferative disease where control of proliferation of latently infected B cells is lostIn HIV patients; oral hairy leukoplakia and lymphomas
Rituximab (anti-CD20 mAb) EBV
Complications: Giant cell pneumonia, encephalitis
Measles Give Human Normal Ig
affects paediatric post-BMT patients. Weekly PCR surveillance. Tx:
Adenovirus
Reduce immunosuppression + Ribavirin
how to test whether it was autoimmune in origin
COOMBS test Haemolytic anaemia
Diagnosis of thalassaemia, sickle cell
Hb electrophoresis
Polycythaemia monitoring
Hct & Hb
Bone marrow response to haemolytic anaemia
Reticulocyte
Joint pain, splenomegaly, skin, nerve and kidney. (in Meeran’s book): cryo means cold. This has more systemic presentation - abnormal proteins deposit all over the body
Associated with Hep C.type III hypersensitivity CryoglobulinaemiaIn patients with cryoglobulinemia, when the blood gets below normal body temperature, it precipitates an abnormal protein that causes the blood to become jelly-like. The thickening of the blood creates damage to the blood vessels and organs throughout the body. This reaction can lead to complications ranging from skin rashes to organ damage/failure, amputations, strokes and even death.
40 year old man with previous hep B. Muscle pain and joint pain. Spares pulmonary capillaries. Renal involvement is prominent feature, thus high BP. associated with Hep B/C. Fever, fatigue, weakness, arthralgia, skin, nerve and, kidney involvement, pericarditis and MI,
Polyarteritis nodosa
EBV, mycoplasma, DAT positive which antibody is involved?
IgM Cold haemolytic anaemia:
DAT test plus jaundice, lymphoma on antibiotics
Warm IgG
MARCH:
MAHA, renal failure, CNS (headaches, hallucinations), haemogobinuria
Young man with fever, leucocytosis and a petechial rash after URTI
HSP
AK2, itching after a bath, myelodysplastic disorder
Polycythaemia vera:
> 20% blasts, Auer rods, Down’s
AML
9;22, myeloid cell
CML
general malaise, weight loss, blurred vision, high IgM
Waldenstrom
should be able to recognize platelets going up with a pneumonia (to around 450 – patient was feeling feverish and unwell)
Infection/Reactive thrombocytosis
platelet count > 600 * 109, both thrombosis (venous or arterial) and haemorrhage can be see, a characteristic symptom is a burning sensation in the hands, a JAK2 mutation is found in around 50% of patients. PMD: DVT, easy bruising Megakaryocyte proliferation results in an overproduction of platelets. T
Essential Thrombocytopenia: platelet count > 600 * 109
tx: hydroxyurea, anagrelide
Can presents as both - bleeding and thrombosis
Middle aged, diagnosed on routine blood tests Massive Splenomegaly, Philadelphia Chromosome. Thrombocytosis is observed in 30% to 50% of patients, occasionally at levels higher than 1,000 x 109/L
CML:
asymmetrical painless lymphadenopathy, hepatosplneomegaly, deaarange LFT worst after alcohol , cyclical fever, Reed-Stenberg Cells. EBV associated. Stage 3 (on both sides of the diaphragm), 4 extranodal signs
Hodgkin’s
Alk1 (liver, BM) t(2;5)
Anaplastic Large Cell Lymphoma
Caribbean and Japanese, HTLV-1 infection, aggressive
Lymphocytosis,hypercalcemia, cloverleaf apperance of the attack cells
Adult T cell leukaemia/lymphoma
Associated with longstanding coeliac disease
Enteropathy-associated T cell lymphoma (EATL)
Associated with mycosis fungoides
Cutaneous T Cell Lymphoma
starry sky, EBV, jaw in African children
Burkitt’s
H pylori and Sjogren’s (parotid)
MALT
The most common cause of testicular mass in 40+,
EBV associated and HHV, eldery men and B-symptoms
Diffuse Large B Cell:
CLL (Smear cells) into lymphoma, name this condition
Richter transformation ( Diffuse Large B Cell)
JC Virus would be present in which lymphoma?
–Progressive multifocal encephalopathy (PML) is a fatal demyelinating disease of the central nervous system (CNS), caused by the lytic infection of oligodendrocytes by a human polyomavirus, JC virus (JCV). PML is rare disease but mostly develops in patients with underlying immunosuppressive conditions, including Hodgkin’s lymphoma, lymphoproliferative diseases, in those undergoing antineoplastic therapy and AIDS.
BCR-‐ABL Translocation
CML (Philadephia Chromosome)
t(15;17)
seen in acute promyelocytic leukaemia (M3)
fusion of PML and RAR-alpha genes
t(8;14
)seen in Burkitt’s lymphoma
MYC oncogene is translocated to an immunoglobulin gene
t(11;14)
deregulation of the cyclin D1 (BCL-1) gene, lymphadenopathy and GI involvement in form of polyps
Mantle cell lymphoma
Patient with back pain, high IgG, plasma cells>20%
Multiple Myeloma
Patient with lump on clavicle and you see a lytic lesion on the Xray. There is also a raised IgA but no other lesions/abnormal calium/anaemia→
Solitary plasmacytoma
A 49-year-old woman presented with a 6-month history of vague aches and pains in her chest. Protein electrophoresis showed a small paraprotein band in the gamma region; this band was an IgG of lambda type. A bone marrow biopsy showed only 12% plasma cells. There was no protein in her urine
Benign paraproteinaemia
Patient with no signs or symptoms and just raised IgG/IgA (can’t remember which!)→ MGUS: raised Ig no symptoms, <10% bast cells, <30g/dl paraprotein
MGUS
10% plasma cells (+ >30g/l) in BM but no CRAB / organ/tissue
Smouldering
IgM paraprotein (weight loss, fatigue, hyper viscosity syndrome, visual)
Waldestrom Hyperglobuinaemia
● 70-something year old, overweight patient with diabetes presents with chronic back pain and mildly raised creatinine. GFR was 55? Paraprotein at 12g/dl
Secondary?- Secondary Amyloidosis is associated with certain infections. The change in renal function is due to basement membrane thickening=Kimmelstiel nodules = Nephrotic syndrome
German woman with recurrent URTIs + UTIs w/ low neutrophils.
Cyclic neutropenia? ELA gene?
Philadelphia 9,22 BCR ABL present. Middle aged person 3% blasts Indolent. This is where Imatinib is the most effective
CML Chronic Pha <5% Imatinib
> 10% blasts in bone marrow. Splenomegaly, lasting up for a year.
CML Accelerated phase.
● Philadelphia 9,22 BCR ABL present, TdT was expressed on cells - 22yr old, 70% blasts! No hepatosplenomegaly. No myelocytes or basophilia. STC treatment
CML in blast phase
Smear cells, elderly asymptomatic patients, generalised lymphadenopathy
CLL
High WBC + CD5 =
CLL
Tartate resistant acid phosphatase –
Hairy Cell Leukemia subtype of CLL
Patient with puritis + hepatsplenomegaly) raised haemoglobin following showering and Jak 2 Mutation:
PRV
elderly, massive splenomegaly, low platelets, red cells, normal white cells
Myelofibrosis (Massive splenomegaly: CML and fibrosis)
associated with DIC (hemmorrhage low platlets) , faggot cell, subtype of AML. ATRA (alpha –trans retinoic acid)
Acute promyelocytic leukaemia
Monoblstic, monocytes, Skin-gum infiltration plus hypokalemia
M4+M5
Pelger Huet cells hyposegmented neutrophil -
MDS
By definition all patients have <20% blasts
myelodysplastic disease
– just the red blood cells are affected. means the condition doesn’t respond to treatment that doctors would normally use for low blood cell counts.
refractory anaemia
the red blood cells, white blood cells and platelets are affected
refractory cytopenia
the red blood cells, white blood cells and platelets are affected, and there’s a higher risk of developing acute leukaemia. Patient presents with recurrent infections (anemia WBC VERY LOW).Pegler huer This can further convert to AML
refractory anaemia with excess blasts (RAEB
What would you see on the iron studies with low iron??
low iron, low ferritin, high TBC, high Transferrin
Thalassaemia trait -
increased hbA2
1st episode DVT or PE, atrial fibrillation (2-3), caridomyopathy, symptomatic inherited thrombophilia, mural thrombus, cardioversion
2.5 if:
recurrent DVT or PE, mechanical prosthetic valve (2.5-3.5), coronary artery graft thrombosis, antiphospholipid syndrome
3.5
Anti Xa assay -
rivaroXaban, fondaparinuX (they have X’s in them)
low in liver disease and in DIC, high in pregnancy
Fibrinogen
breakdown product of fibrin
D dimers
Unfractionated heparin:
monitor APTT
Warfarin
PT
doesn’t need monitoring except late pregnancy and renal failure. Also aspirin.
LMWH (Clopidrogel):
When after the surgery patient comes back to the baseline risk of clotting?
DVT post surgery - will be back to baseline risk after 3 months of treatment
Pt with AF - 2.5
Leave Warfarin the same (2-3)
Pt with prosthetic valve - 2.5-3.5
Leave Warfarin the same (2-3)
Someone with a prosthetic valve
Between 3.0 and 4.0
Pt with previous DVT 4
Decrease Warfarin
Pt with previous DVT 1.5
Increase Warfarin
INR 5-8 minor bleeding
Vitamin K
Major bleeding:
prothrombin complex concentrate
Patient has prolonged APTT, normal PT and normal bleeding time
Haemophilia A
prolonged APTT, normal PT and prolonged bleeding time.
vWF
increased INR, APTT alcoholic
vitamin K deficiency
One question the woman had a clot but so did her father and sister familial very clotty
ATIII deficiency.
for pregnant woman with seizures and schistocytes but
TTP because of fever & neuro symptoms etc… so not DIC (tricky Q).
can get skin necrosis if given warfarin
Protein C problems
10 year old with recurrent episodes of jaundice + mild anaemia - blood film shows NO central pallor, RBCs are spherical
hereditary spherocytosis
10 year old afro-caribbean girl, history of chest crises and dactylitis, normal Hb with positive sickle solubility test
Sickle trait
● 10 year old caucasian who had been on a holiday, presented with low platelets (thrombocytopenia) and profound anaemia (6g/dl + oliguria) - anaemic blood film showed ?bite cells, and schistocytes (MAHA)-
G6PD
How would platelet defect would present?
Increased bleeding time
What does heparin increase?
INR, bleeding time, APTT
What would be increased in Haemophila?
APTT
What would be increased in vW?
APTT and bleeding time
If you see the reticulocytes what would you think?
Aplastic anemia
Causes of microcytic anemia
FAST: Ironn, ACD, sideroblastic, Thalassemia
incusions following splenectomy
Howell-Jolly Bodies
Cabor rings are characterisitc feature of whaT?
Megaloblastic Anemia
substittiution mutation which predisposes to DVT, the most common in the caucasia population
prothrombin G20210A
frothy sputum following transfusion
fluid overload
centrocytes and centroblast lymphoma
follicullar
Hypocelluular bone marrow and macrocytosis
Macrocytosis results from the relase of the fetal haemoglobin in attempt to compnase for RBC
Mechanism behind the anemia of chronic disease
IL-6 released from macrophages increases hepcidin, which retains iron in the macrophages,
alcohol- what sort of anemia
non-megaloblastic macrocytic anemia , also sideroblastic
Target cells can be found
LITH Liver disease Iron Deficency Thalssaemia Hyposplenism
two weeks after viral ilness child develop a purpuric rash
ITP - self limiting ilness caused by preformed IgG binding to virus coated RBC (type II)
What is underlying mechanism of the DIC?
malignancy, placent abruption, activate the clotting cascade leading to the secretion of the Tissue Factor
Also Plasmin is heavily involved
Whchich cells contribute to granulomatous disease>
Monocytes
What other causes of monocytosis do you know?
SLE, Brucelliosis, Typhoid, VZV, CMML
Eosinophilia causes
Parasytic, asthma, autoimmune, Lymphomas (Hodkin and non-Hodkin). NSAID
infection, cold day, jaundice, dark urine
Paroxymal Cold Haemoglobinurias
defect in red cell membrane, leading to intravscular haemolysis, PNH (pancytopenia, NEw Thrombus, hemolytic anemia)
Flow cytometry would be diagnostic with this patient.
prognostic factor melanoma
beta 2 microglobulin
what is the main complication of massive transfusion?
thrombocytopenia
/In what ways alcohol causes low platlets?
supresion of Bone Marrow
the most common lymphoma, waxing and weaning lymphadenopathy
Follicular
exploded popcorn kernel, peripheral lymphadenopathy without the B-symptoms
Nodular lymphocytic Hodkin
Haptoglobin is increased or decreased in intravasculara haemolysis?
Decreased (marker of haemolytic anemia)
Megacariocytes with excess blasts and hyperlobulated nuclei
MDS with 5q deletion
