Haematology Flashcards by Federico Ciardi

what is the management of haemophilia B?

factor IX replacement

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what grade is follicular NHL?

(aggressive/variable/indolent)

indolent

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treatment duration of 1st VTE with unknown cause?

3-6 months warfarin, possibly lifelong

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how long to you give maintenance treatment for ALL in different patient groups?

girls and adults - 2 years

boys - 3 years

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what is the poor outcome in CLL?

reichter transformation into DLBCL

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managing elevated INR

>8, no bleed/minor bleed

Stop warfarin.

Vitamin K (oral/IV) no bleeding/if risk factors for bleeding or minor bleeding.

Check INR daily

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what life-threatening complication is associated with APML

DIC

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what cells stain +ve for CD15/30?

Hodgkin, Reed-Sterberg cells

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what is the main difference between von willibrand disease and haemopihlia VIII on clotting studies?

both aPTT is normal

bleeding time is increased in VWD

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what is the treatment for waldenstrom macroglobulinaemia?

plasmapheresis

chlorambucil, cyclophosphamide (& other chemo)

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which inherited BM failure presents with renal malformations, microopthalmia and skeletal abnormalities?

fanconi anaemia

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what is the diagnostic definition of accelerated phase CML?

>10% blasts in BM/blood

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what is paraprotein?

A monoclonal immunoglobulin which appears as a dense narrow band (M band) on electrophoresis, seen in multiple myeloma

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what is leukoerythroblastic anaemia?

5 features

immature cells from myeloid lineage (myelocytes/normoblasts) on blood film

features include:

poikilocytes
nucleated RBCs (normoblasts)
reticulocytosis
myelocytes/promyelocytes
thrombocytopenia

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what is the lymphocyte count diagnostic of CLL?

>4.0 x10^6/mL

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what infections give a monocytosis?

brucellosis, typhoid fever, tuberculosis, EBV/CMV, infective endocarditis, malaria, Chagas/ASS, visceral leishmaniasis, rickettsial infection

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EBV-induced Burkitt lymphoma is responsive to what chemotherapy?

cyclophosphamide

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The patients blood film shows classic Pelger-Huet neutrophils and the bone marrow biopsy reveals 15% blasts.

Diagnosis?

Refractory aneamia with an excess of blasts.

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what is the treatment for chronic ITP?

IVIG, steroids, splenectomy

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over-expression of cyclin D1

mantle cell lymphoma

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Neutrophilia with visible toxic granulation and vacuoles on the blood film. suggests…

acute fungal infection

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A 58 year old lady complains of lethargy and “easy bruising”. She presents with purpura. Her FBC reveals Hb 10.5g/dl; WBCs 2.3x109/l and platelets 8x109/l. Blood film reveals <1% Blasts, and marrow aspirate shows 20% dysplasia in erythroid lineage, 60% dysplasia in platelet lineage, 5% dysplasia in granulocyte lineage, and less than 5% blasts.

refractory cytopenia with multilineage dysplasia

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which conditions give raised ESR and low CRP?

SLE, pregnancy, multiple myeloma, anaemia, lymphoma

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which are the very aggressive NHLs?

Burkitt and ALL

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what are the defective red cells seen in MDS?

ringed sideroblasts

what is the current treatment for hodgkin lymphoma?

**ABVD** adriamycin/doxorubicin bleomycin vinblastine dacarbazine/procarbazine

what is the diagnositic criteria for smouldering myeloma and what is the treatment?

EITHER serum monoclonal protein \>30 g/L OR plasma blasts \>10% no CRAB symptoms or findings Do not treat - no affect on mortality

what is the treatment for follicular lymphoma?

Rituximab CVP

a patient with long standing rheumatoid develops nephropathy and amyloid is found in the renal biopsy. what classification of amyloidosis is like likely to be?

secondary amyloidosis

which inherited BM failure has hepatic failure, short stature, endocrine abnormalities and pancreatic dysfunction?

Schwachmann-Diamond syndrome

what blood test reflects the acitvity of fibrinogen?

thrombin time

which immunoglobulin is commonly raised in MM?

IgG

what are the features that count as B symptoms in hodgkin lymphoma?

night sweats fever weight loss \>10% in 6/12

which cytokine is important growth factor in the development of multiple myeloma?

IL-6

psot-transfusion: diarrhoea and maculopapular rash/skin necrosis...

GvHD most commonly in immunosuppressed patients

t(11;14)

mantle cell lymphoma

what is the stardard prophylactic ajdunt to MTX therapy? what is the adjunt for high-dose or intrathecal MTX?

folate leucovorin

A 65 yr old man presents to his GP with general malaise, weight loss and visual disturbances that he likens to looking through a watery car windscreen. On examination he has peripheral lymph node enlargement. Protein electrophoresis shows an IgM paraprotein.

Waldenstrom macroglobulinaemia

benzene is a risk factor for what?

Acute leukaemia

between LMWH and UFH, which is more likely to induce HIT?

UFH

what is the use of anti-lymphocyte/thymocyte globulin?

use in the setting of acute organ rejection animal antibodies vs T cells, depletes the immune response

Anti-proliferative used in polycythaemia rubra vera and essential thrombocythaemia

hydroxyurea

what circulating serum proteins can be used to determine the severity of MM? (i..e NOT the monoclonal antibody, which is used to diagnose)

high beta-2 microglobulin & low albumin part of the ISS staging system

which inherited BM failure is a pure red cell aplasia?

Diamond-Blackfan syndrome

what is Cooley's anaemia?

beta-thalassaemia major

hodgkin lymphoma accounts for what %age of all lymphoma?

20%

what is the difference in specificity and sentitivity of pernicious anaemia antibodies?

sensitive - anti-parietal cell (\>90%) specificity - anti-intrinsic factor, as 10% of healthy people have anti-parietal cell

which malignancy has the highest likelihood of promoting thombosis?

pancreatic CA - expresses high levels of tissue factor

in patients with cancer and acute venous thromboembolism, which is the most effective drug at reducing the risk of recurrent VTE?

LMWH

what is the management for haemophilia A?

desmopressin (increases vWF) no IM injections or NSAIDs factor VIII replacement

what is the treatment for myelofibrosis?

hydroxycarbamide, thalidomide, steroids and SCT

other than imatinib, what are the meds useful in CML?

hydroxyurea and IFN

following massive transfusion, what are the considerations for platelets?

replacing with packed red cells and FFP will not replenish plts expect plts = 50 x10^9/L after 20 U blood (= 2 blood volumes) if \<50-75 (depends on local guidelines) must transfuse plts as well

Used in solid organ transplantation, multiple sclerosis, NHL. Depletes B lymphocytes more than T lymphocytes.

Rituximab (anti-CD20)

a causative agent in hodgkin lymphoma?

EBV

for which diseases may splenectomy be useful treatment?

**Th**e **PIIES** thalassaemia, pyruvake kinase def, immune haemolytic anaemia, immune thrombocytopenic purpura, eliptocytosis, spherocytosis

which are the indolent NHLs?

follicular and marginal

treatment of recurrent VTE?

lifelong warfarin

management of rised INR 5-8, no bleeding

withhold few doses. reduce maintenance restart when under 5

what medication is given as thrombotic prophylaxis in DIC?

LMWH

what is the clotting picture of vW disease?

elevated aPTT elevated bleeding time decreased VIII and vWF normal PT, plts

what is the treatment for mantle cell lymphoma?

rituximab CHOP

what is anagrelide?

use in treatment of essential thrombocytosis when: * \> 60 years * plts \>1,000 x10^9/L * history of thrombosis inhibits maturation of plts from megakaryocytes

t(14;18)

follicular lymphoma

chondrocalcinosis is commonly found in which diseases?

hypercalcaemia (hyperPTH), gout, arthritides, haemochromatosis, possibly post-trauma

what is the diagnostic definition of CML?

\<5% blasts in the bone marrow/blood maybe slowing increasing over time

A 10 year old boy presenting with tan-to-grey hyperpigmented patches on his upper trunk, neck and face, nail ridges and leukoplakia.

dyskeratosis congenita

what are the abnormal plts seen in MDS?

micromegakaryocytes with hypolobated nuclei

what biologic can be used in the treatment of all non-Hodgkin T cell lymphoma?

alemtuzumab (Campath, anti-CD52)

what biologic agent used in NHL, solid tumour transplant and multiple sclerosis depletes lymphocytes and gives Graves/AITP as side effects?

Campath (alemtuzumab, anti-CD52)

what is the diagnostic criteria for multiple myeloma?

* M-protein in serum ≥30g/L and/or bone marrow clonal plasma cells ≥10% * Requires 1 or more of the following: 1. Calcium elevation (\>2.75 mmol/L) 2. Renal insufficiency (creatinine \>176 mmol/L) 3. Anaemia (Hb \<10 g/dL or 2 g/dL below the lower limit of normal) 4. Bone disease (lytic or osteopenic)

how quickly do you expect transient abnormal myelopoesis in a newborn to resolve?

a couple of weeks

what are the blood findings in haemophilia A?

APTT elevated PT normal Factor VIII assay decreased

what is the the first line mangement for MM? how do you catagorise these patients?

suitable for auto-SCT: lenalidomide + dex unsuitable for auto-SCT: melphalan + pred + thalidomide

managing elevated INR major bleeding

stop warfarin give prothombin complex concentrate (if available), or FFP IV Vitamin K

what is the numerical definition of lineage displasia? what is the definition of refractory cytopenia with multilineage dysplasia?

dysplasia in \>10% of cell lineage at least 2 myeloid lineages are dysplastic with bi-/pan-cytopenia in peripheral blood

what is the treatment for CLL?

FCR fludarabine - purine analog, inhibits DNA synthesis cyclophosphamine - DNA crosslinks, leading to apoptosis rituximab - anti-CD20, kills B cells

which are the aggressive NHLs?

DLBCL and mantle cell

what race most frequently carries the prothrombin G20210A mutation?

caucasians (5%)

explain a normal G6PD assay in a patient with G6PD deficiency

if the assay is taken immediately during/after an acute challenge the bone marrow will have initiated erythropoesis to compensate therefore newly produced G6PD will give a false negative in the assay. The assay should be done at a later date to confirm the diagnosis.

TRAP +ve WBCs in serum suggest...

hairy cell leukaemia

what is the treatment for DLBCL?

Rituximab-CHOP

treatment duration of 1st VTE with known cause?

3 months warfarin

which of the following is a immunohistochemical marker for multiple myeloma? * CD19 * CD20 * CD138 * Surface Ig

CD 138

what is Evan's syndrome?

CLL associated with autoimmunity - AIHA and ITP

CNS and testicular involvement are common in which disease?

ALL

managing elevated INR 5-8, minor bleeding

Stop warfarin. Vit K slow IV. Restart when INR \<5

what is the staging system for CLL?

Binet staging high WCC, \<3 nodes - stage A high WCC, \>3 nodes - stage B anaemia or thrombocytopenia - stage C

which are the important CLL prognostic factors?

Zap 70 Immunoglobulin gene configuration (mutated vs. unmutated) cytogenetics (trisomy 12, del 13q, del 17p)

what is the dose of LMWH for treatment of DVT/PE

175 U/kg until INR in therapeutic range

what T lymphocyte marker is abberently expressed on malignant B cells in chronic lymphocytic leukaemia?

CD5

deletion of long arm chromosome 5 what is it and what is the treatment?

a MDS usually found in elderly women lenolidomide - really good response

which inherited BM failure presents with skin discolouration, oral leukoplakia and nail dystrophy?

dyskaratosis congenita

what are the abnormal white cells seen on MDS?

hypogranulation/hyposegmented (pseudo-Pelger-Huet anomaly)

which innate immune cell drives the response to *salmonella typhi?*

monocytes

lymphadenopathy and HSM elevated WBCs hypercalaemia predisposing viral infection

adult T-cell leukaemia HLTV-1 from Japan or Carribean

what age group get ALL and AML?

children get ALL adults get AML (and under 2s)

what is the second line management for MM in both groups of patients?

suitable for auto-SCT: bortezomib + dex unsuitable for auto-SCT: melphalan + pred + bortezomib