1 mbchb v: imaging haematology session 2 mj coetzee

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MBChB V: ImagingHaematology

Session 2

MJ Coetzee

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Table of contents

• Haematological malignancies– Leukaemias

• Acute• Chronic

– Myeloma– Lymphoma

• Thrombosis & haemostasis– Thrombocytopenia– Haemophilia– DIC

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Leukaemias: rough classification

• Myeloid (granulated cells)– Chronic

(myeloproliferative conditions)

• e.g. Chronic myeloid leukaemia (CML)

– Acute myeloid leukaemias

• arise suddenly, deadly• e.g. acute

promyelocytic leukaemia

• Lymphoid (lymphocyte line)– Chronic

• e.g. Chronic lymphocytic leukaemia

• Course: years

– Acute lymphoblastic• arise suddenly, deadly• e.g. acute

lymphoblastic leukaemias (ALL)

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LymphoidMyeloid

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More mature cells: chronic leukaemias

More immature

cells: acute leukaemias

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Myeloproliferative diseases

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Organomegaly in a

patient with a

myeloproliferative

disease

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CMLChronic Myeloid

Leukaemia

Peripheral blood:neutrophils with left shift & eosinophils &

basophils

Increase in white cell layer in centrifuged blood:

“leukaemia”

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CML:Philadelphia chromosome t(9;22)

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Target of Gleevec (drug against CML): works on signal transduction

• The normal tyrosine kinase transmits a signal from the surface to the nucleus in a controlled manner

• The abn. tyrosine kinase is an ONCOGENE that stimulates the nucleus to divide

Intracelllularsignaltransducers

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Polycythaemia verapolycythaemia = many cells in the

blood;vera = Latin for true (unexplained)

Patient ofSir William Osler in whom he first

described PV

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Many RBCs on a blood smear

Cellular biopsy in PV

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CT of oganomegaly

Splenomegaly

Bone marrow fibrosis

Idiopathic myelofibrosis

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Essential thrombocythaemia

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Gangrene in essential

Thrombocythaemia

(abnormal platelet clumping)

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Acute myeloid leukaemia Different classifications

FAB (French-American-British) WHO (world Health Organization)

Can represent different cell lines Myeloid cells of stages of maturation Promyelocytes Erythroblasts Platelets, etc.

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Myeloblasts: peripheral blood

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Myeloblasts with an Auer rod

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AMLBone

marrow packed with

blasts

Myeloblasts with granules (granules

are typical of myeloid cells

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Lymphoidleukaemias

• Chronic lymphocytic leukaemia

• Acute lymphoblastic leukaemia

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CLL (chronic lymphocytic leukaemia): clinically

Cervical lymph nodes

Mediastinal lymph nodes

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CLL bone marrow: infiltrates

of mature lymphocytes

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CLL: peripheral blood with many lymphocytes

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CLL: laboratory

CLL cells express typical antigens op their membranes. These are detected by means of flow cytometry

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ALL: clinical picture

• Rapid course• Lymphadenopathy• Organomegaly• Haemorrhages• Infections

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ALL: lymphoblasts

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Burkitt's lymphoma/leukaemia blasts

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Myeloma

• Vague complaints– Bone pain– Fractures– Infections– Malaise

• Please remember to determine paraproteinsin blood and urine

• 1% of cancers• > 10 % of

haematologic cancers in the US

• Annual incidence ± 4 per 100,000

• Increase in cases– ?enhanced availability

and use of medical facilities

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Monoclonal band of abnormal Ig

Normal

Myeloma

Normal

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Myeloma

•Osteopenia

•Lytic lesions

•Pathological fractures

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Myeloma: rouleaux on peripheral blood

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Myeloma: plasma cells in bone marrow

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Congested veins resulting from paraproteins, esp. IgM

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Haemostasis

• Haemorrhagic diatheses– Hereditary haemorrhagic diatheses

• Haemophilia• Von Willebrand disease

– Acquired haemorrhagic diatheses• ITP

• Thromboses– Venous– Arterial

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Purpura(fine

peticchiae)

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Purpura (ecchymoses): larger bleeds

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Haemarthrosis in haemophilia

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Haemophilia: X-rays (ankylosis)

Knee

Elbow

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Haemophilia Family tree

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Haemophilia: repeated bleeds

Dark hypertrophic synovium; osteoartritis;osteophytes

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Dangerous bleeds

Bleed in floor of the mouth stretches downward and

obstructs the airways

Volkman contracture or compartment syndrome of fore arm (hand useless)

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ITP: bone marrow packed with megakaryocytes

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TTP: few platelets red cell fragments(always exclude HIV)

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Superficial thromboses: clinicallySkin necrosis

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DIC: clinical picture

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DIC: histological picture