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Senior Academic Half Day: Malignant Haematology. Beth Harrison Department of Haematology University Hospitals Coventry and Warwickshire NHS Trust. Normal haematopoiesis Investigations in malignant haematology Approach to a patient with pancytopenia Diagnosis and management. Case 1.

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Senior academic half day malignant haematology

Senior Academic Half Day:Malignant Haematology

Beth Harrison

Department of Haematology

University Hospitals Coventry and Warwickshire NHS Trust


  • Normal haematopoiesis

  • Investigations in malignant haematology

  • Approach to a patient with pancytopenia

  • Diagnosis and management


Case 1
Case 1

  • 35 year old male

  • 6 weeks recurrent throat infections

  • 2 weeks easy bruising

  • Hb 8.6

  • WCC 1.2

  • Platelets 12

Pancytopenia – he will need a bone marrow examination





Case 11
Case 1

  • 35 year old male

  • 6 weeks recurrent throat infections

  • 2 weeks easy bruising

  • Hb 8.6

  • WCC 1.2

  • Platelets 12


  • Hb 8.6

  • WCC 1.2 +

  • Platelets 12

    =

    Acute Leukaemia


What is acute leukaemia?

What is a “blast”?



Case 12
Case 1

+

+

Diagnosis = Acute myeloid leukaemia

Bone marrow failure

Blasts in bone marrow (+blood)

Molecular diagnostics


Case 13
Case 1

  • The Patient receives some chemotherapy

  • Presents to A&E

  • Pyrexial

  • Shivery, vomiting, diarrhoea



Neutropenic sepsis1
Neutropenic Sepsis

  • Treat as neutropenic without waiting for FBC result

  • Blood cultures

  • Broad spectrum antibiotics within 30 minutes of presentation

  • IV fluid resuscitation

  • Get help



Management of acute leukaemia
Management of acute leukaemia

  • Chemotherapy

  • BUT:

    • Filtered air

    • No plants or flowers

    • No unnecessary visitors

    • Washed food – no salad or grapes or black pepper

    • Antifungal prophylaxis

    • Mouthcare


Indications for bone marrow
Indications for bone marrow

  • Diagnostic

    • Abnormal FBC

    • Investigation of paraproteinaemia

    • Bone lesions in pelvis accessible by this route

    • Pyrexia of unknown origin

      • ? TB in HIV+

      • ? foreign travel / splenomegaly

    • Isolated splenomegaly with diagnosis unclear from PB

  • Staging

    • Hodgkin Lymphoma / Non Hodgkin Lymphoma

  • Treatment response

    • Leukaemia, Myeloma, Lymphoma etc


Case 2

  • 56 year old man

  • back pain, vomiting and constipation

  • Na 145 Calcium 3.25 K 5.7 Total protein 126 Urea 46 Albumin 34 Creat 565

  • Hb 8.7


Investigations:

  • Protein electrophoresis – of what?

  • Bone marrow examination – for what?

  • Skeletal survey – is what?


Investigations:

  • Serum / urine electrophoresis

  • Bone marrow examination

  • Skeletal survey


What is the diagnosis
What is the diagnosis?

  • Multiple myeloma

  • First management issues?

  • Correct calcium

  • Give fluids


Renal failure in myeloma
Renal Failure in Myeloma

  • Light chain deposition in kidney

  • Hypercalcaemia

  • Hyperuricaemia

  • Dehydration

  • Non-steroidal anti-inflammatories

  • Plasma cell infiltration of kidney


Urine free light chains an old story
Urine free light chains: An old story

Previous polyclonal antisera against light chains could not distinguish light chains bound into whole immunoglobulin molecules from free light chains


Case 3
Case 3

  • 35 year old woman with 2 years of lethargy and intermittent LUQ pain

  • now complaining of dizziness

Visible white cells


Case 31
Case 3

  • On examination:

  • Massive splenomegaly Fundal haemorrhages

  • Diagnosis

  • Chronic myeloid leukaemia with hyperviscosity resulting from WCC

  • Immediate management

  • Get the white cell count down!!


Myeloproliferative disorders
Myeloproliferative Disorders

  • Clonal, pre-leukaemic

  • Uncontrolled proliferation of one or more bone marrow lineages:

    • Red cells – primary polycythaemia

    • Platelets – essential thrombocythaemia

    • White cells (myeloid) – chronic myeloid leukaemia

    • Fibroblasts - myelofibrosis


Myeloproliferative disorders1
Myeloproliferative Disorders

  • Primary Polycythaemia and Essential Thrombocythaemia:

    • Increased vascular events

    • Treatment is aimed at reducing these

Hb>19?

Plts>700?

Ask!


Causes of hyperviscosity
Causes of hyperviscosity

  • Paraprotein (IgM > IgA > IgG)

  • High WCC (CML / AML > CLL)

  • High red cell mass (polycythaemia)

  • Raised platelet count

    • (>1,000, myeloproliferative rather than reactive)


Causes of splenomegaly
Causes of splenomegaly

  • Haematological

    • Chronic myeloid leukaemia, Myelofibrosis

    • Chronic lymphatic leukaemia

    • Acute lymphoblastic leukaemia

    • Lymphoma (various)

  • Infective

    • EBV

    • Chronic malaria

    • Visceral Leishmaniasis

  • Liver Other

    • HCV / HBV with portal hypertension

    • Any cause cirrhosis with portal hypertension



Indications for lymph node biopsy
Indications for lymph node biopsy

  • Generalised lymphadenopathy, FBC unhelpful.

    • (Also palpable cervical LN with mediastinal LN on CXR)

  • Isolated lymphadenopathy – no obvious pathology in the anatomical region drained

    • (ENT: nasendoscopy NAD, FNA unhelpful)

  • Regional lymphadenopathy with obvious primary pathology inaccessible to biopsy


Findings on lymph node biopsy
Findings on lymph node biopsy?

  • Reactive

  • Necrotic

  • Granulomatous – TB, Sarcoid?

  • HIV?

  • Metastatic Carcinoma

  • Metastatic Melanoma

  • Lymphoma


Non-Hodgkin’s Lymphoma: T cell

Hodgkin Lymphoma

Non-Hodgkin’s Lymphoma: B cell


Case 41
Case 4

  • Nodular Sclerosing Hodgkin Lymphoma


Case 42
Case 4

Treatment:

Chemotherapy

Radiotherapy

  • Risks of treatment:

    • Breast cancer

    • Thyroid cancer

    • Secondary leukaemia / myelodysplasia

    • Infertility

    • Other endocrine failure - early menopause

    • Bones

    • Cardiac damage (chemo + radiotherapy)

Risks of treatment?





Indolent non hodgkin lymphoma localised to one site
Indolent Non-Hodgkin Lymphoma: localised to one site




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