haematology cases Flashcards by Emily Southall

summarise where myeloblasts usually are

<5% is normal in marrow
5-10% = myelodysplasia in marrow
>20% AML

never in peripheral blood - if there it is likely AML or leucoerythroblastic

may havbe Auer rods - AML

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summarise where lymphoblasts usually are

they are precurser lymphoid cels - never in peripheral blood

<5% is normal in marrow
>20% - ALL

lymphoblasts are TdT +ve - no Auer rods

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2 causes of low MCV

iron deficiency
haemoglobinopathies

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2 causes of mod high MCV

alcohol
hypothyroidism
combined iron and folic acid deficiency

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2 causes of super high MCV

B12 def
folate def

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what are the b cell markers and what are they used for

CD19 - epitope for CAR T cells - used for lympoblastic leukaemia or lymphoma
CD20 - epitope for rituximab moab - chemo for lymphoma, and rheumatoid

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what are the T cell markers

CD3 - all mature
CD4 (helper)
CD8 (cytotoxic)
CD5 - normal in peripheral blood, not in B cells

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markers of lymphoid differentiation ie maturity

TdT - marker of immature T and B lymphoblasts - ie immature
* surface Ig marker of matyre B cels and plasma cells

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differentiate these features for myeloma and normal

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causes of raised polyclonal Ig

HIV, infection, SLE

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how to approach interpreting blood results

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haemolytic anaemia - because of hx likely SLE with autoimmune haemolytic anaemia
Borderline raise in MCV - because reticulocytes around

BR - want to know if conjugated or unconjugated

LDH released from haemolysing red cells

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haemolytic anaemia and get these results

Liver is normal othe than raised unconjugated BR - so pre-hepatic jaundice

LDH released from haemolysing red cells

spherocytes - smaller than normal erythrocytes and no central pallor

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causes of spherocytes

hereditroy spherocytosis

autoimmune haemolytic anaemia

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where is the defect for inherited haemolytic anaemia

defect of the red cells

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where is the defect for acquired haemolytic anaemia

the env/toxins in env

red cell is normal - although morphological damage may occur

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causes of hereditory haemolytic anaemia and how you would diagnose them

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causes of acquired haemolytic anaemia and how you would dx them

non immune eg malaria, damage from metal valve, drugs, MAHA

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Anaemia of inflammation
Normal HbA2 excludes beta thal trait - HbA2 would be raised

Ferritin is an inflammatory protein

Transferrin is normal

differentiate anaemia of inflammation (ACD) from IDA

transferrin is the key - will be high in IDA, and low/normal in ACD

ferritin low = confirms IDA, but can be high if also have inflammation - because it is an inflammatory protein

summarise anaemia of chronic disease

anaemia if inflammation- mediated by high hepcidin levels

body iron stores are sequested and unavailable for erythropoeisis
high hepcidin inhiobits GI absorption of iron and sequesters it in macrophage and kuppfer cells

hepcidin is an anti-bacterial/inflammatory response protein - removing iron from blood deprives bacteria of iron needed for proliferaton

causes of isolated single lineage cytopenia with otherwise normal FBC

often poeripheral destruction/shortened survival
* immune destruction
* non immune - infection (malaria), mechanical (DIC), consumption sequestration (splenomegaly)

failure of production
* haematinics - iron only required for Hb - isolated anaemia. B12 and folate needed for DNA synth - can affect all lineages
* drugs - agranulocytosis

B12 deficiency
Get pancytopenia because B12 needed for DNA synth - so get reduced in all lineages

Whereas iron is only required for haem synthesis - gives isolated anaemia- no impact on anything else

Organ specific autoimmune conditions - if have one organ specific autoimmune disorder likely to get another one eg pernicious anaemia

MCV would also be high in haemalytic anaemia because of the reticulocytes - woulndt be this high

Unconjugated BR - because ineffective red cell production in bone marrow -> more death -> high unconjugated BR

Leucoerythroblastic anaemia
Due to prostate cancer met to bone marrow

Pancytopenia
Myeloma and leukaemia don’t usually cause these sx - urine and haematuria

Leucoerythroblastic blood film - tear drop red cell

Precursers which are present - nuceated red cells - should only be present in the marrow

summarise pancytopenia

For philidelphia chr - with rtPCR or cytogenetics Healthy person Chronic myeloid leukemia - Leukaemia with excess prolifeation - ie a myeloproliferative disorder, proliferation with retained maturation Retained maturation WCC very high - much higher than would expect in any type of infection film - Heterogenous - All mainly myeloid

summarise CML

Hx - lethargy/hypermetabolism/thrombotic event: monocular blindness CVA, bruising, bleeding o/e - massive splenomegaly +- hepatomegaly FBC - Hb and plts preserved/raised, massive leucocytosis blood film - neutrophils and myelocytes (not blasts if chronic phase), basophilia

how can we use leukaemia markers in treatment

use rtPCR to see ratio of cells with just abl gene vs those with bcr-abl

Imatinib - abl kinase inhibitor

Transform to become acute leukaemia - refractory or resisntant to drugs - acquired new mutations and is a blast crisis

JAK2V617F mutation analysis Pruritis withheat - typical of polycythaemia vera

what result would you expect

expression of CD5 in B cell population

what is the likelu dx and why

CLL Leukocytoiss All leukocytes Rest are preserved - favours chronic Normal mature lymphocytes Smudge cells - artefact

cell based prognostic factors for CLL

IgHV mutation status CLL FISH cytogenetic panel TP53 mutation status (Chr 17p del and/or TP3 point mutation)

clinical staging system for CLL

Binet or Rai CLL IPI score based on lymphocytosis, lymphadenopathy, pancytopenia

clinical staging system for CLL

Binet or Rai CLL IPI score based on lymphocytosis, lymphadenopathy, pancytopenia

what are the clinical issues with CLL

what is B cell targetted therapy

Bruton's X linked agammaglobinopathy (inherited condition) * abnormal B cell tyrosine kinbase gene - pre B cells cannot develop to mature B cells * abscence of mature B cells * No circulating Ig after 3 mo **ibrutinib** tries to mimic this condition - inhibit bruton tyrosine kinase

what is ruxolotinib

JAK2 tyrisine kinase inhibitor

treatment options in CLL

Myeloma Riased IgA Immune paresis Low hb - bone marrow infiltration by plasma cells

problem with dx myeloma

a lot of sx are general complaints of the elderly -> late dx can be catastrophic - spinal cord compression or established renal failure

No Havent exclude dlight chain only myeloma - don’t impact full IgG or IgA - need to check serum free light chains

Super high serum free light chain - high level of circ light chain - pass trhough basement membrane of kidney -> **cast nephropathy**

blood - O rhD -ve *Can give men and postmenopausal women RhD +ve - would sensitise but only relevant in pregnancy* FFP - AB RhD-ve

Use cryoprycippitate It is the fibrinogen that is low