Haemotology of Systemic Disease Flashcards

Question

What are signs of MAHA?

Answer 1

Red cell fragments (microangiopathy) Low PLT DIC (fibrin deposition/ degradation)/ bleeding may be linked to underlying adenocarcinoma

Answer 2

Polycythaemia vera (PV) - clonal myeloproliferative disorder (JAK2 mutation) Secondary Polycythaemia (Raised EPO)

Answer 3

Cancer: HCC, bronchial and renal cancer Oxygen related: High altitude, hypoxic lung disease, cyanotic heart disease

Answer 4

Increase/ decreased Mature or immature Cell type Lineages (red, white, platelets)

Answer 5

Inflammatory \> Auto immune \> Infective Malignant Genetic Metabolic

Answer 6

Granulocytes Monocytes T lymphocytes B lymphocytes NK cells

Answer 7

Blasts Promyelocytes Myelocytes

Answer 8

FBC/ blood film HIgh WBC/ look at lineage/ morphology/ maturity

Answer 9

corticosteroids underlying neoplasia tissue inflammation (e.g.colitis, pancreatitis) Pyogenic infection myeloproliferative/ leukaemic disorders infection

Answer 10

Localised and systemic infections acute bacterial, fungal, certain viral infections Some infections characteristically do not produce a neutrophilia e.g. brucella, typhoid, many viral infections.

Answer 11

Presence bands, toxic granulation, and signs of infection/inflammation

Answer 12

Neutrophilia basophilia plus immature cells myelocytes, and splenomegaly. Suggest a myeloproliferative (CML) Neutropenia plus Myeloblasts (AML)

Answer 13

Parasitic infestation allergic diseases e.g. asthma, rheumatoid, polyarteritis,pulmonary eosinophilia. Underlying Neoplasms, esp. Hodgkin’s, T-cell NHL (reactive eosinophilia) Drugs (reaction erythema mutiforme)

Answer 14

Eosinophils part of the “clone” FIP1L1-PDGFRa Fusion gene

Answer 15

Rare but seen in certain chronic infections and primary haematological disorders TB, brucella, typhoid Viral; CMV, varicella zoster sarcoidosis chronic myelomonocytic leukaemia (MDS)

Answer 16

Neutrophils: bacterial Eosinophils: Parasitic Basophils: Pox viruses Monocytes: Chronic (TB/ brucella)

Answer 17

N: AI disease, Tissue necrosis E: Allergic (Asthma, atopy, drug)

Answer 18

Neutrophils: all types Eosinophils: Hodgkins/ NHL

Answer 19

Neut: CML Baso: CML Monocytes: CMML

Answer 20

Neut: CML Baso: CML Monocytes: CMML

Answer 21

Secondary (reactive); polyclonal response to infection, chronic inflammation Primary; monoclonal lymphoid proliferation e.g. CLL, NHL. Is it T or B cell?

Answer 22

Approach Clinical Symptoms suggestive of infection or lymphoma Massive lymphadenopathy or splenomegaly FBC Degree of lymphocytosis Light microscopy/ morphology Mature cells or primitive lymphoblasts Flow cytometry Lineage; B or T cells Stage of differentiation Molecular genetics Rearranged: T cell receptor or Immunoglobulin gene

Answer 23

1. Infection (EBV, CMV, Toxoplasma, viral hepatitis, rubella, herpes infections) 2. Autoimmune disorders 3. Sarcoidosis

Answer 24

Mature lymphocytes (PB) reactive/atypical lymphocytes (IM) small lymphocytes and smear cells (CLL/NHL) Immature Lymphoid cells in PB lymphoblasts (Acute Lymphoblastic Leukaemia)

Answer 25

\>3.5x109/l

Answer 26

Monoclonal antibody conjugated to fluorescent dye Anti-CD4/blue, CD34/green, CD7/purple

Answer 27

Forward scatter: Size Side scatter: granularity

Answer 28

Germline/inherited (gene mutated): Factor IX ▪ Deficiency \> haemophilia B ▪ Excess \> FIX Padua (gene therapy) Erythrocytes ▪ Deficiency\> b globin chain production \> b Thalassaemia ▪ excess \> VHL gene \> Chuvash polycythaemia

Answer 29

Erythrocytes ◼ Excess \> hypoxia \> cyanotic heart disease ◼ Deficiency\> anti-RBC antibodies \> Immune haemolysis Factor VIII ◼ Excess \> inflammatory response ◼ Deficiency\>anti-FVIII auto-antibodies (acquired haemophilia A)

Answer 30

Soluble factors: ◼ raised FVIII in Inflammation \> Thrombosis risk. Erythrocytes ◼ Raised {altitude/hypoxia or Epo secreting tumour} ◼ Reduced BM infiltration or deficiency disease {Vit B12 or Fe} Shortened survival {Haemolytic anaemia} Platelets ◼ Raised {Bleeding, Inflammation, splenectomy} ◼ Reduced BM infiltration or deficiency disease {Vit B12 } Shortened survival {ITP, TTP} Leucocytes ◼ Raised {Infection, Inflammation, corticosteroids} ◼ Reduced BM infiltration or deficiency disease {Vit B12 }

Answer 31

Somatic/acquired (gene mutated) BM rapid turnover organ! Erythrocytes ▪ Excess\> JAK2 V617F\> Polycythaemia vera Myeloid/granulocytes ▪ BCR-ABL1 \> Chroinic myeloid leukaemia

Answer 32

60: 40 kappa and lambda 99: 1 kappa or lambda skewed

Answer 33

\>**Morphology**- blood film/ biopsy- architecture of tumour, cytology, cytochemistry \>**Immunophenotype**- flow cytometry, immunohistochemistry \>**Cytogenetics**- conventional karyotyping, fluorescent in-situ hybridisation _(FISH)_, Interphase FISH, Metaphase FISH \>**Molecular genetics**- mutation detection, direct sequencing, Pyrosequencing, _PCR analysis_, gene expression profiling, _whole genome sequencing_

Answer 34

Primitive lymphoid blast cells expressing B cell marker \> B cell Acute lymphoid leukaemia Mature lymphoid cells expressing T cell antigens and involving skin\> cutaneous T cell lymphoma Mature erythrocytes with JAK2 mutation\> polycythaemia vera

Answer 35

◼ Factor VIII \> Haemophilia A\> bleeding ◼ Protein C \> pro-thrombotic

Answer 36

Polycythaemia Anaemia

Answer 37

Granulocytes \> leukaemia(CML) or reactive eosinophilia Lymphocytes \> leukaemia(CLL) or Lymphopenia (HIV)

Answer 38

Essential thrombocythemia or ITP

Answer 39

Primary disorders arise from DNA mutation(s)

Answer 40

Secondary disorders are changes in haematological parameters 2ndry to a non-haematological disease

Haemotology of Systemic Disease Flashcards

(65 cards)