Haematology Flashcards
IDA
Microcytic
Incr TIBC + transferrin
Decr ferritin
Anisocytosis/raised RDW/ poikilocytosis
Vit B 12 deficiency
Macrocytic megaloblastic anaemia
Aplastic anaemia
Low: Hb, reticulocytes, neutrophils, platelets, BM cellularity
High MCV due to HbF release
CD8 destruction of BM -> fatty changes
80% idiopathic
10% dyskeratosis Congenita + fanconi anaemia
10% secondary: SLE, drugs: carbamazepine, chloramphenicol, anticonvulsants, radiation
Anaemia of chronic disease
Microcytic hypochromic anaemia + rouleax
Decr TIBC + serum iron
Incr ferritin
IL 6 mediated -> liver hepcidin production
Beta thalassemia
Microcytic anaemia Iron studies normal Target cells Minor= 1 beta globin chain Major= 2 beta globin chains Severe anaemia, req reg transfusions, skull bossing hepatosplenomegaly
Chronic alcohol consumption
Non megaloblastic macrocytic anaemia
Chronic renal failure
Normocytic normochromic anaemia
Reduced EPO secretion
Lead poisoning
Microcytic anaemia
Basophilic stippling
Pappenheimer bodies
Causes of MAHA
TTP HUS DIC SLE Schistocytes aka helmet cells, incr BR, jaundice
Paroxysmal nocturnal haematuria
Haemolysis
Haemoglobinuria
Ham’s test +ve
Myelofibrosis
Tear drop cells / dacrocytes Circulating megakaryocytes BM aspirate dry, bloody tap Pancytopenia Hepatosplenomegaly - extra medullary haematopoiesis Abnormal MKcytes release PDGF + TGF beta
Megaloblastic anaemia
Cabot rings
Hyper segmented neutrophils
Haemophilia A
F VIII deficiency
Low APTT
ITP/ AITP
2 wks after viral illness in children
Self limiting
Type 2 hypersensitivity reaction vs GpIIb/IIIa
ITTP
MAHA Fever Renal failure Fluctuating CNS signs Haematuria Low platelets ADAMTS13 mutation -> vWF multimers -> platelet thrombi Req plasma exchange
Glanzmann’s
GpIIb/IIIa mutation
Causes coagulation
Haemophilia B
Factor IX deficiency
Osler Weber Rendu
Aka hereditary haemorrhagic telangiectasia
AD
Telangiectasia on skin + mucous membranes
= epistaxis + GI bleeds
Bernard soulier syndrome
GpIb mutation
Receptor for VWF in clot formation
Prothrombin G20210A
Inherited thrombophilia
Guanine -> adenine
= amplification of PT production
Predisposition to DVT + PE
Factor V Leiden
AD inherited thrombophilia Arginine -> glutamine Impaired degradation of FV by protein C Incr risk DVT + miscarriage Test function of APC
Antiphospholipid syndrome
AI thrombophilia
Anti cardiolipin + lupus anticoagulant
Bind to phospholipids on cell surface -> trigger Coag cascade
Incr risk DVT + miscarriage
Buerger’s
Aka thromboangitis obliterans
Small + med vasculitis of vessels of hands + feet
Assoc with smoking
Claudication
Gas gangrene + amputation
Corkscrew appearance of arteries on angiogram
Protein C or S deficiency
Inherited thrombophilia / rarely : warfarin / liver disease
Impaired degradation of FVa + FVIIIa
Incr risk DVT
Anti thrombin deficiency
Inherited thrombophilia
Usually inhibits FII (thrombin) + FXa
Transfusion complications:
Fluid overload
Dyspnoea
Incr JVP
Pink frothy sputum
Due to too fast transfusion rate >2ml/kg/hr
Or in those with pre-existing cardiac or renal failure
Transfusion complications:
GVHD
Donor lymphocytes attack recipient cells
Especially skin + GIT
Diarrhoea, macpap rash, skin necrosis
Irradiate donor blood to reduce risk
Transfusion complications:
Anaphylaxis
Incr risk in those with IgA deficiency
Due to IgA in donor blood
Transfusion complications:
TRALI
Acute non cardiogenic pulmonary oedema within 6 hrs
Dry cough, dyspnoea, fever
Anti WBC Ab in donor blood attack host leukocytes
Donor has had sensitising event e.g. Transfusion/transplant
Transfusion complications:
Immed haemolytic transfusion reaction
Within 1-2 hrs post transfusion
ABO incompatibility
Abdo + loin pain, vomiting, facial flushing, haemoglobinuria
Host IgG + IgM -> donor RBCs = removed by retic endothel syst
Transfusion complications:
Febrile non-haemolytic reaction
After pregnancy
Antileukocytic Abs form
Low fever rigors
Transfusion complications:
Fe overload
In cases of recurrent transfusion: thalassemia SCD
Bronzed skin
Short stature
Heart failure
Transfusion complications:
Bacterial infection
High fever, rigors, hypotension
Hep B, hep C, HIV
Transfusion complications:
Delayed haemolytic transfusion reaction
> 24 hrs post transfusion
Milder than immed haemolytic reactions
Hairy cell leukaemia
CLL subtype Middle aged men Hair like projections on tumour cells CD25 (IL2-R) + CD 11c Tartrate-resistant acid phosphatase: TRAP Splenomeg, hepatomeg, pancytopenia
CML
Elderly Elevated: neutrophils, basophils, eosinophils BM: hyper cellular T(9;22) BCR-Abl Imatinib
AML
BM: >20% myeloblasts blasts Ayer rods t(8;21) FLT3 gene CD13, CD33, CD34 Seen in Downs
CLL
Smudge CeLLs
elderly men
>4000 cells per microlitre
Small lymphocytes in lymph nodes with irregular nuclei
Mixed with larger prolymphocytes -> aggregate to proliferation centres
Hypogammaglobulinaemia
Reiter’s transformation -> DLBCL
ALL
Paediatric
BM: >20% lymphoblasts
APML
AML M3 subtype
Faggot cells: loads of Auer rods
PML-RARA
ATRA asap to prevent DIC
Adult T cell leukaemia/lymphoma
Assoc with HTLV1
Endemic in Japan + Carribean
Clover leaf nuclei in ATL cells
LNpathy, hepatsplenomeg, skin lesions, hypoCa
Large granular lymphocytic leukaemia
Blood: large lymphocytes
BM: azurophilic granules
T cell prolymphocytic leukaemia
Aggressive T cell leukaemia
Inv Chr 14 (q11;q32)
Burkitt’s lymphoma
Latent EBV, most prevalent in Africa children + teenagers C-Myc Chr 8 Starry sky Endemic: mandibles mass Non endemic: abdominal mass
Hodgkin’s lymphoma
EBV -> NF-kappaB activation
Reed Sternberg cells (binuc/multinuc surrounded by eosinophils)
Painless asymmetrical LNpathy, except with ETOH
Fluctuates in size
Pel-Ebstein fever
B sx
Extra nodal involvement rare
Mantle cell lymphoma
Elderly men
t(11;14)
Bcl1 + Ig heavy chain
Overextension cyclin D1
Follicular lymphoma
Middle aged t(14;18) Over expression Bcl2 Centrocytes, centroblasts Non aggressive but difficult to cure
Diffuse Large B Cell Lymphoma
Elderly Large lymphocytes with diffuse pattern of growth Immunodef - assoc with EBV Body cavity based - assoc with HHV8 Follicular lymphoma can transform CLL can transform - Reiters
Mycosis fungoides
Cutaneous T cell lymphoma
Elderly men
Rash like lesions
Appear similar to eczema/psoriasis
Angio centric lymphoma
Cutaneous mass in nasal area
Tumour cells express NK cell markers
Oft infected with EBV
Essential thrombocythaemia
High platelet count >600
Bleeding + thrombosis
BM: hyper cellular giant platelets, MKcyte clustering, hyperplasia
Rx: hydroxyurea, anagrelide
Multiple myeloma
BM: >10% plasma cells, multi nuc, prom nucleoli
Rouleaux
IgG, IgA, para proteins
Paraprotein bands > 30g/L on electrophoresis
Incr ESR + Ca, punched out lytic lesions on XR (n ALP)
Bench Jones proteins in urine
Polycythaemia Rubra Vera
JAK2 gene, exon 2, V167F
Incr Hb low EPO
Gout = incr cell turnover
Headaches, dizziness, stroke = hyperviscosity
Plethoric, pruritic after baths, peptic ulcer = hyper mast cell degran
Hereditary spherocytosis
AD haemolytic anaemia Dysfunct: spectrin/ankyrin/ band 4.2 \+ve osmotic fragility -ve Coombs Jaundice, splenomeg, pigment gallstones \+ve fhx
Hyposplenism
Howell Jolly bodies Target cells Uncleared RBCs Lymphocytosis Macrocytosis Acanthocytes: speculated RBCs
Precautions to take in Hyposplenism
Penicillin V prophylaxis
Pneumococcal conjugate vaccine
Hib vaccine
Meningococcal vaccine
Diseases in which splenectomy may be required
Thalassemia Pyruvate kinase deficiency AIHA ITP Ellipto + spherocytosis
Cold AIHA
IgM mediated 28-31 Celsius Causes: CLL Lymphoma Mycoplasma EBV Idiopathic
Paroxysmal cold haemoglobinuria
AI haemolytic anaemia
Children following infection
Donate Landsteiner antibodies
IgG antiglobulins at low temperatures
Smouldering myeloma
Paraprotein >30g/L
Clonal plasma cells >10%
No myeloma related organ/ tissue impairment
MGUS
Serum paraprotein
Combined Polycythaemia
Aka smokers Polycythaemia
Carbon monoxide -> incr EPO = incr red cell mass
+ smoker’s reduced plasma volume
Marginal zone lymphoma
Chronic antigen stimulation -> malig transformation
B cell NHL
Extranodal: MALT= stomach, lung, thyroid, breast, synovium, lac, saliv
Nodal:
Splenic:
Nodular lymphocytic HL
Non classical Lymphocytic + histiocytic Reed Sternberg variant 'popcorn cells' Children or 30-40yrs More common in Afro Carribean Peripheral LNpathy without B sx
Nodular sclerosis classical HL
Nodular growth pattern fibrous bands separating nodules
Mixed cellularity classical HL
Heterogenous group
Diffuse/ vaguely nodular pattern without band forming sclerosis
Lymphocytic rich HL
Nodular growth pattern
Background infiltrate: mainly lymphocytes
Lymphocyte depleted HL
Diffuse growth pattern
Hypocellular
Lack of inflammatory cells