Cytopenias - National University Hospital · - Fever - Chills - Rigors - Hypotensive ... NO...

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Cytopenias The what, why and how Dr Esther Chan Associate Consultant Haematology

Transcript of Cytopenias - National University Hospital · - Fever - Chills - Rigors - Hypotensive ... NO...

Cytopenias The what, why and how

Dr Esther Chan

Associate Consultant

Haematology

3 main cell lines

• RBC/Haemoglobin

• White cell counts

• Platelets

The WHAT

Anaemia

Leucopenia

• The only clinically relevant parameter is neutropenia

• Risk is of severe infection and this correlates to absolute neutrophil count

• ANC

– <1-0.5: Significantly increased risk of infection

– <0.5: Highest risk of infection

Thrombocytopenia

• Generally not clinically significant if >100K

• Concern of bleeding increases once Plt<30k

Summary: The What

• Drop fm pt’s baseline

• Neutrophils <1

• Platelets <100 (<30)

The WHY

How is the FBC measured?

• Haematology Analysers

• Principle of WBC, RBC and Plt counts

– Cells forced in single file through aperture

– Causes momentary decrease in electrical current

– Creates pulse

• Amplitude proportional to size

• Number of pulses proportional to number

• Note that most modern machines combine laser, impedance, radiofrequency, direct current, peroxidase staining to optimize sensitivity

However…. • Machine errors are still possible!

The WHY - Lesson 1

• Exclude spurious low counts!

Important Approach

• Concept of

– Production issues

• Empty

• Packed

• Faulty

– Peripheral consumption/

destruction

‘Empty’

• Aplastic anemia/ Marrow hypoplasia

Causes: -idiopathic causes, - viral infections, - drug related causes, (Chemotherapy) -RT etc.

‘Packed’

• By marrow infiltration with abnormal cells

– hematological malignancies ie leukemias

– non hematological malignancies (metastasis)

• By marrow fibrosis

– Myelofibrosis

Myelodysplastic Syndrome

• Defined as acquired bone marrow disorder

• Characterised by ineffective haematopoiesis

• Proliferation of abnormal clone of cells. which replaces normal haematopoietic cells.

• Clinical manifestation of BM failure as well as tendency to transform into acute leukaemic phase

• May be primary or secondary to other causes eg. chemotherapy, radiotherapy or environmental toxins.

‘Faulty’

Peripheral destruction/consumption

• Infections

– Dengue

• Autoimmune

– SLE

• Hypersplenism

– Cirrhosis with splenomegaly

The WHY - Lesson 2

• For approach to cytopenias

– Is it

• Production problem?

• Destruction problem?

– Narrows down differentials

The HOW

On seeing cytopenias on the FBC…

• Do not interpret an FBC by itself!

1. Need for guidance from clinical history & physical examination

2. Need to take cues from the FBC/PBF

Clinical History

• Symptoms/ Signs:

- Fever - Chills - Rigors - Hypotensive - Toxic

- Malar rash - Arthritis

- Alcohol history Hypersplenism as cause of cytopenias - Splenomegaly

Sepsis as cause of cytopenia

Possible SLE with concomittant cytopenias

Signs/Symptoms

Systemic Symptoms - LOA - LOW - Fever

Clinical Signs - HEPATOMEGALY - SPLENOMEGALY - LN SWELLING - PALLOR

Possible Haematological Malignancy

• Leukaemia • Lymphoma

Cues on the FBC/PBF

• ‘Empty’ Marrow

– Pancytopenia

– No abnormal cells on the PBF

– No early white/red cells

• ‘Packed’ Marrow – Leucoerythroblastic picture

• Early RBC/WBC in the peripheral blood

• Tear drop cells

– Blasts

– Abnormal lymphoid cells

– Rouleux

• ‘Faulty’ marrow

– Dysplastic features

• RBC: Anisopoikilocytosis, Basophilic stippling

• WBC: Hypo/Hyper-granulated forms

• Plt: Plt anisocytosis, Hypogranular forms

Other factors

• Other cell lines

– Monocytopenia? Bicytopenia? Pancytopenia

• Differential Counts

Real life examples

• Case 1

• Describe the FBC

• What else would you want to know?

• What are the differentials?

Don’t forget!

• Clinical History

– Bruising

– Loss of weight

– Tired

– Fever

• Physical Examination

– Lymph nodes palpable

Initial Impression

• Leuocytosis with reduced Hb/ plts

• Associated with systemic symptoms.

Differentials:

- Sepsis (Viral)?

- Hematologic malignancy?

Other Factors:

• Final diagnosis

– Acute leukaemia

Case 2

• How do you want to proceed?

• Clinical History

– 50 year old man

– Loss of appetite and some loss of weight.

– No bleeding complications.

– Noted increased lethargy at home.

– Admitted when noted Hb low and thrombocytopenia.

– NO hypothyroidism symptoms.

– NO history of liver disease

– NO special drug use

– PE: NO hepatosplenomegaly

Other factors

• PBF findings

– Hyposegmented Neutrophils seen

• Diagnosis

– Myelodysplastic syndrome

Case 3

• Any thoughts?

• Clinical history

– 50yr Female

– Elective admission for Total knee replacement

– Incidental finding

• What to do next?

– Cancel operation?

– Bone Marrow Aspiration?

• Other factors

Case 4

• Clinical History

– 30yr Female

– Brought to A&E by family

– Fever for 3 days

– Drowsiness, unable to rouse from bed for 1 day

• Diagnosis

– Thrombotic Thrombocytopenic Purpura

Case 5

• History

– 60yr Male

– Well with no complaints

– FBC done as part of health screening by his company

• Other factors

Classification of anemia – MCV and reticulocyte count

Microcytic

MCV

Normocytic Macrocytic

Low retics:

Iron Deficiency anemia

Sideroblastic Anemia

Normal/high retics:

Thalassemias

Low retics

Normal WBC/Platelets:

• AOCD

• Early IDA

• Renal failure

• Pure red cell aplasia

Pancytopenia

• Primary failure: AA

• Secondary failure: chemo/RT, MDS

Low retics:

Megaloblastic anemias

Non-megaloblastic:

• Liver disease

• Alcohol

• Hypothyroidism

• Drugs

• MDS

High retics:

Reticulocytosis

Summary – The HOW

• Correlate FBC with

– pt ‘s clinical picture

– & other factors (PBF/Differential counts)

• To evaluate and manage all cytopenias safely

The End! Thank you for your attention

Email: [email protected]