Leukaemia Flashcards
What is leukaemia?
Leukaemia is a progressive, malignant disease of the blood-forming organs, characterised by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow.
How can leukaemia be classified?
Acute or chronic, according to the degree of cell differentiation (not the duration of disease).
Myelogenous or lymphocytic, according to the predominant type of cell involved (myeloid or lymphoid)..
Aetiology of leukaemia
The exact cause is unknown
Signs and symptoms of leukaemia
Fatigue Weight loss Fever Pallor Ecchymoses Petechiae Dyspnoea Dizziness Palpitations Bleeding Recurrent infections Each sub-type has its distinguishing features.
Which tests are essential for the definitive diagnosis of leukaemia?
Definitive diagnoses often require bone marrow biopsy and/or blood analysis.
Types of leukaemia
Acute lymphocytic leukaemia (ALL) Chronic lymphocytic leukaemia (CLL) Acute myelogenous leukaemia (AML) Chronic myelogenous leukaemia (CML) Hair cell leukaemia (HCL)
What is ALL?
A malignant clonal disease that develops when a B/T-precursor-stage lymphoid progenitor cell becomes genetically altered through somatic changes and undergoes uncontrolled proliferation.
Which type of leukaemia is the most common in paediatrics?
Acute lymphocytic leukaemia (ALL) is the most common leukaemia in paediatrics, accounting for up to 80% of leukaemias in this group and 20% of leukaemias in adults
Signs and symptoms of ALL
Most cases present with signs and symptoms associated with cytopenias. Symptoms of anaemia include: fatigue weakness shortness of breath poor concentration dizziness or feeling lightheaded cold hands and feet Symptoms of leukopenia include: frequent infections fever Symptoms of thrombocytopenia include: bleeding and bruising easily difficulty with stopping bleeding internal bleeding
What is the initial cause of seeking medical attention in a patient with ALL?
Enlarged lymph nodes
What is CLL?
A cancer of B lymphocytes. Failure of B lymphocytes to undergo maturation and full differentiation leads to a monoclonal population of dysfunctional but self-renewing B lymphocytes.
Which organs does CLL infiltrate most commonly?
These lymphocytes can infiltrate lymphatic tissues and haematopoietic organs such as the liver, spleen and bone marrow.
What is a key risk factor in the development of CLL?
Age over 60 years
What are the key diagnostic factors in a patient with CLL?
Lymphadenopathy, splenomegaly (in 50% of cases), and shortness of breath and fatigue
Which tests are essential for the definitive diagnosis of CLL?
It is diagnosed by FBC with differential, blood smear showing smudge cells, and flow cytometry.
Most cases are diagnosed on a routine FBC for an unrelated reason.
What is AML?
The clonal expansion of myeloid blasts in the bone marrow, peripheral blood, or extra-medullary tissues.
Who is a typical patient with AML?
Occurs predominantly in older adults.
Common signs and symptoms of AML
Common findings are pallor, ecchymoses and petechiae.
What is acute promyelocytic leukaemia?
Acute promyelocytic leukaemia (APML) is a form of acute myelogenous leukaemia with distinct cytological and clinical features, which has specific management.
What is CML?
A malignant clonal disorder of the haematopoietic stem cell that results in marked myeloid hyperplasia of the bone marrow.
What are the other names for CML?
May also be called chronic granulocytic leukaemia, chronic myelocytic leukaemia, or chronic myeloid leukaemia.
Who is a typical patient with CML?
Median age at presentation is around 53 years, and the only known risk is exposure to ionising radiation.
Common signs and symptoms of CML?
Many cases are asymptomatic but possible symptoms include fever, chills, malaise, weight loss, and night sweats. Around 75% of patients have splenomegaly.
Which tests aids the definitive diagnosis of CML?
All patients will have an elevated WBC count.
Diagnosis should be confirmed by the presence of the Philadelphia chromosome and/or the BCR-ABL rearrangement in peripheral blood or bone marrow cells.
What is the blast crisis?
Blast crisis refers to the transformation of chronic myelogenous leukaemia (CML) from the chronic or accelerated phase to the blast phase.
Common signs and symptoms of blast-phase CML
Anaemia, infections, abnormal bleeding, bone pain, and constitutional symptoms (night sweats, weight loss, fever) are common presenting complaints of blast-phase CML.
What is the goal in a patient with blast-phase CML?
The goal is to achieve a complete haematological remission or at least return to the chronic phase in order to perform stem cell transplant.
What is HCL?
A B-cell malignancy characterised commonly by symptoms of fatigue, a markedly enlarged spleen and a distinctive histological appearance on peripheral blood smear and bone marrow biopsy.
What are the most important characteristics of HCL?
Hairy cell leukaemia (HCL) is characterised by B-cells with delicate cytoplasmic projections resembling hair.
There is marked geographic variation in the frequency of HCL (also known as leukaemic reticuloendotheliosis) and it is relatively uncommon.
Who is a typical patient with HCL?
The median age of patients is 50 years and HCL affects more men than women.
It often presents with abdominal discomfort or fullness.
What is the abdominal discomfort in HCL attributed to?
This is attributed to splenomegaly, which is present in around 60% to 90% of patients.
Is HCL curable?
The disease is not curable. However, it is highly responsive to therapy and maybe managed successfully for a decade or more.
How can leukaemia cause pancytopenia?
Leukaemias may cause pancytopenia through decreased production, or increased destruction or sequestration, of blood cells.
The main complication of neutropenia
Neutropenia contributes to some of the typical symptoms of leukaemia, including recurrent infections.