Haematology in systemic disease Flashcards
Haematological disorders are as a result of excess/deficiencies in….
- Soluble proteins
2. Cellular haematology
How can you tell if a haematological disorder is primary or secondary?
Primary is a problem in the bone marrow/blood due to DNA mutations. They are inherited (haemophilia B) or acquired (CML).
Secondary = healthy bone marrow/blood with alteration due to non-haematological disease.
What laboratory findings point towards iron deficiency anaemia?
- > Microcytic Hypochromic Anaemia
- > Reduced Ferritin
- > Reduced Transferrin Saturation
- > Increased Transferrin
- > Increased TIBC - Total Iron Binding Capacity
What are causes of iron deficiency anaemia?
Fe deficiency is bleeding until proven otherwise.
In Pre-menopausal women, it is often menorrhagia
In Men/Post-Menopausal women it can be more sinister i.e.
-> GI Cancer - Gastric/Colonic/Rectal
-> Urinary Tract Cancer - Renal Cell Carcinoma, Bladder Cancer
How is the diagnosis of Leuco-erythroblastic anaemia made?
Abnormal presence of immature cells in peripheral blood.
- Tear drop poikilocytes
- Nucleated RBC
- Myelocytes (immature myeloid cells)
What are causes of Leuco-erythroblastic anaemia?
All of the causes are due to the INFILTRATION OF THE BONE MARROW.
- > Malignant (most important)
i) Haematopoetic i..e leukaemia/lymphoma/myeloma
ii) Non-Haemopoietic i.e. metastatic breast/bronchus/prostate
-> Myelofibrosis
this is the fibrosis of the bone marrow in response to primary haematological cancer
-> Severe Infection
miliary TB, severe fungal infection
What are the clinical features common to ALL haemolytic anaemias?
- > Anaemia
- > Reticulocytotis
- > Unconjugated Bilirubin Raised (pre-hepatic)
- > LDH raised
- > Thrombocytopenia
What are causes of haemolytic anaemias?
- Inherited (primary)
- > Membrane i.e. Hereditary Spherocytosis
- > Cytoplasm/Enzymes i.e. G6PD Deficiency
- > Haemoglobin i.e. Sickle Cell Disease/ Thalassaemia - Acquired; (secondary) Coombs Test.
i) Immune Mediated (DAT+ve)
Typically seen in systemic diseases involving the immune system
-> Malignancy e.g. Lymphoma/CLL
-> AutoImmune e.g. SLE
-> Infection e.g. Mycoplasma
-> Idiopathic
ii) Non-Immune Mediated (DAT-ve)
- > Infection i.e. Malaria
- > Microangiopathic Haemolytic Anaemia (MAHA), seen in Underlying Adenocarcinoma’s and Haemolytic Uraemic Syndrome.
Coombs Negative Film presenting with fragmentation and thrombocytopenia…
MAHA
Coombs positive with spherocytes…
And why are spherocytes produced?
Immune mediated haemolytic anaemia
Antibodies stuck to RBC membrane. Reticulo-endothelial system removes parts of membrane with antibodies and RBC becomes round.
How does Microangiopathy cause haemolysis?
Inappropriate coagulation in blood vessels
Platelet activation
Fibrin deposition
Red cells fragmented when driven through
Bleeding (due to low platelet and coag factors)
What are causes of neutrophilia?
i) Reactive/Infection
Neutrophilia + Toxic Granulation and no immature cells
Seen in
- > Pyogenic i.e. Pus Releasing Infection
- > Steroids
- > Underlying Neoplasia
- > Tissue inflammation i.e. colitis/pancreatitis
ii) Malignant
Neutrophilia + Basophilia + Immature cells. Suggests a CML
Neutrophilia + Myeloblasts. Suggests AML
Seen in Myeloproliferative/leukaemic disorders.
What causes eosinophilia
i) Reactive Eosinophilia
- > Parasitic infestation
- > Allergic diseases e.g. asthma, rheumatoid, polyarteritis, pulmonary eosinophilia.
- > Neoplasms, esp. Hodgkin’s, T-cell NHL (reactive eosinophilia)
- > Drugs (reaction erythema multiforme)
ii) Chronic Eosinophilic Leukaemia - VERY RARE.
- > Eosinophils part of the “clone”
- > nFIP1L1-PDGFRa Fusion gene
What causes monocytosis?
Very Rare
i) Chronic Infections
- > TB, Brucella, Typhoid
- > Viral - CMV, VZV
- > Sarcoidosis
ii) Primary Haematological Disorders
- > Chronic Myelomonocytic Leukaem
What are causes of a reactive lymphocytosis?
- > Infectious Mononucleosis (EBV)
- > CMV
- > Toxoplasmosis
- > Infectious Hepatitis, Rubella, Herpes
- > AutoImmune
- > Sarcoidosis
