Hameatology - Main

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Haematology Overview

• Haematology is the study of blood, blood-related disorders, and malignancies

Anaemias

• Anaemia is defined as low level of haemoglobin in the blood for the age, gender and ethnic background of an individual.
• Defined by low haemoglobin (Hb) due to decreased red cell mass
• To diagnose, note levels through full blood count and treat with supplements to ensure normal function
• Normal Hb range, Female: 110-180 g/L, Male: 130-180 g/L
• Check Hb levels and blood films, tests for any underlying issues and make sure that levels are at a normal
• Check iron levels as well for issues
• Haemolysis in the blood, and infections are common concerns
• Hb levels are different in males and females, due to menstruation

WHO Definition of Anaemia

• Males: Hb < 13g/dL
• Females: Hb < 12g/dL

Anaemia Classifications

• Based on red blood cell size, anaemia as the following subtypes:
• Microcytic: Under 80 fL
• Normocytic: 80-100 fL
• Macrocytic: Over 100 fL

Normal Full Blood Count (FBC) Values

• Key ranges for blood components of a typical, healthy individual
• Male haemoglobin: 130-180 g/l
• Female haemoglobin: 110-180 g/l
• Haematocrit: 0.35-0.50
• Mean Corpuscular Volume (MCV): 77-87 fl
• Mean Corpuscular Haemoglobin (MCH): 25-33 pg
• White cell count: 4.5-10 x 10^9/l
• Platelets: 150-400 x 10^9/l

Normal MCV

• 80 to 100 fL

Normal Haematocrit

• Male: 40-50%
• Female: 36-44%

Full Blood Count

• Determines Hb level, MCV reduced when microcytic is present
• Tests detect High reticulocyte count as signs of haemophilia

Terminology

• Specialized terms utilized in haematology to describe cell counts
• Cytosis: Too many cells
• Paenia: Not enough cells
• Thrombocytosis: Too many platelets
• Thrombopaenia: Not enough platelets

Main Types of Anaemia

• Anaemia has differing subtypes:
• Microcytic Hypochromic: Iron deficiency related
• Normocytic: Anaemia of chronic disease (rheumatoid arthritis, chronic kidney disease)
• Macrocytic: B12 and Folate Deficiency related

Iron Deficiency Anaemia

• Common causes should be investigated during exams
• Common causes, Dietary deficiency, Malabsorption of iron, Chronic blood loss, GI tract issues, Menorrhagia
• Hypochromic Microcytic Anaemia: Pale and shrunken red blood cells, can be caused by low ferritin levels
• Iron deficiency anaemia is low Hb due to low iron levels
• Most common type of Anaemia

Iron Deficiency Anaemia Causes

• Further causes related to iron deficiency anaemia:
• Blood loss
• Poverty
• Diet low in iron
• Malabsorption related to Crohn's, ulcerative colitis, coeliac's
• Heavy periods
• Pregnancy
• Loss of blood

Iron Deficiency Anaemia Symptoms

• include Pale skin

B12 and Folate Deficiency

• Deficiencies can result here
• The body stores folate for 4 months, found in leafy vegetables, liver, and fruit
• The body stores B12 for 2-6 years, found in animal protein
• B12 absorption necessitates Intrinsic Factor in the terminal ileum
• Absorption occurs in the small intestine; those with Crohn's may be deficient here
• Can also be caused by pregnancy

Folate Deficiency

• Reduced intake, especially in the elderly, and alcoholism, are common causes

B12 Deficiency

• Inadequate intake can be a factor
• Pernicious Anaemia causes Intrinsic factor/antibodies against parietal cells
• Low gastric acid (10-30% of partial gastrectomy patients) can be a symptom
• Abnormal absorption in the terminal ileum may be a symptom of autoimmune (AI) condition

Pernicious Anaemia

• Malabsorption of vitamin B12 results from decreased Intrinsic Factor
• Linked to thyroid, Vitiligo autoimmune issues, and Addison's disease
• Often the Lack of intrinsic factor is needed from parietal cells needed to absorb b12
• Commonly caused by autoimmune conditions, or immune system
• Reduces Hb levels (macrocytic)

Pernicious Anaemia Symptoms

• Fatigue
• Paraesthesia
• Glossitis
• Pallor

Diagnosing B12 and Folate Deficiency

• Assess Red cell folate and serum and Vitamin B12 levels
• Intrinsic Factor Antibodies should be tested for

Treating B12 and Folate Deficiency

• Identify underlying cause and replace orally (folate) or by lifetime injection of Vitamin B12

Diagnosis of B12 Deficiency

• FBC: Low Hb, low serum B12
• Macrocytic anaemia: Large red blood cells, smear, screening
• Autoantibodies against gastric parietal cells/intrinsic factor

Vitamin B12 treatment

• increase b12 consumption by Red meat
• Take b12 supplementation through, injections, or B12 tablets

Folate deficiency anaemia

• Anaemia (low levels of Hb) due to low levels of folate where folate is present in leafy/dark, green vegetables

Folate deficiency anaemia causes

• Include, pregnancy, inadequate diet, alcoholism, and anti-folate drugs

Folate deficiency symptoms

• Can be the same of general anaemia

Folate deficiency diagnosis

• Include a FBC with Low Hb, folate levels,
• And Macrocytic anamia, smear

Folate Deficiency Treatment

• Includes increase in leafy and green vegetables (increase folate), or Folic acid tablets

Anaemia

• Consider anaemia in cases of bleeding

Other Causes of Macrocytic Anaemia

• Drug-related, such as methotrexate, and alcohol, are related to the above causes
• Myelodysplasia is related to bone marrow function

Blood Transfusions

• For transfusions, plasma is separate from red blood cells
• Avoid transfusions where possible to prevent cross-infection
• Transfuse after blood loss of 70 G/L or if symptomatic
• Match to the recipient's blood group
• Rhesus negative is the 'universal donor', and and AB positive the 'universal recipient'

Neutrophils

• Neutrophils are bacterial
• Neutrophil count can increase in bacterial infection, trauma or after surgery
• Decreases in viral infections or from some drugs

Lymphocytes

• Lymphocytes, they increase with CLL and viral infections
• Lymphocytes, can decrease with steroid treatments, SLE

Eosinophils

• Eosinophil count can increase in some allergic disorders

Platelet Abnormalities

• Normal platelet count: 150-400 x 10^9/l
• A platelet count of <50 is an issue
• Platelets are also involved in bleeding to check for signs of blood loss, such as a PT/INR
• Abnormal time is 3-8 minutes

Thrombocytopenia

• A platelet count of less than 50 is an issue

Diagnosis (von willebrand)

• Full w/h is needed including any signs and symptoms Activated aPTT and Thromboplastin time
• The level of Vw factor and the degree of that factor

Petechiae

• Platelet abnormalities may relate to petechiae on skin
• A platelet count of less than 50 is an issue
• Spontaneous Bleeding occurs when the counts fall below 20
• Thrombocytopenia is the most common cause, as well as Disseminated Intravascular Coagulation (DIC)

Immune Thrombocytopenia

• ITP, often seen in major trauma settings
• Recent infection or infectious mononucleosis are causes
• Bone marrow infiltration or leukaemia can be causes, as well as acute leukaemia

Sickle Cell Anaemia

• These are autosomal recessive conditions and the most serious type of cell disease
• Leads to sickled blood/RBC shape on blood film and have a lower life average and a variety of issues which can be diagnosed, caused by abnormal haemaglobin
• Can treat with Chelation These are what Leads to sickled blood, and causes infections as well
• Homozygote = disease state
• Heterozygote = trait
• On exposure to decreased Oxygen tensions loading may cause infarction
• Shape is unoptimized for carrying O2 resulting in death of tissue and MI
• May lead to loss of limbs

Sickle Cell Anaemia Potential Triggers

• Includes: Anemia/Impaired growth, Being vaso-occlusive, And triggers such as infection, dehydration and changes in weather
• Also be cautious of a general anesthetic and hypoxia, as they are very high risks in this condition

Diagnosing Sickle Cell Anemia

• Test through pregnancy during Newborn screening
• FBC screening shows low Hb, with high reticulocyte
• And abnormalities in Hb electrophoresis

Sickle Cell Anaemia Factors

• Positive family Hx
• From Sub-Saaharan Africa these signs
• These signs and Symptoms start from a few months old

Sickle Cell Anaemia Treatment

• Treatment helps with reducing crises that involve infections and dehydration through, for example, warm clothing and fluid control

Sickle Cell Anaemia Considerations

• General anesthetic, be wary of fasting and temperature
• Make sure to administer, full extra oxygenation can reduce effects of lack of oxygen in the blood.

Thalassaemias

• Thalassaemias have differing disorders, Mediterranean disease and auto dominant inherited disorders; characterized by decreased haemoglobin production (either alpha or beta globin chains) .
• Decreased the production of one or more globin chains, Alpha and Beta types have decreased Alpha and Beta Chain
• Anaemias that lead to, Decreased O2 retention the blood cells
• Thalassaemias Symptoms Factors are; Chronic anemia (fatigue, pallor) and Iron overload because off blood transitions
• Microcytic problems due to lack of B cells caused it Blood transitions help to relieve anemia
• Can test through test screens or haemoglobin electrophoresis

Microcytic

• Caused by lack of B cells

Sickling/Thalassaemia

• Care should be taken due to decreased Oxygen Tension, with specific attention during Sedation/GA

Leukaemia

• Leukaemia is cancer of blood cells and its type depends on cells affected (Bone Marrow and Lymphatic system)
• Most often seen in adults, is very common, in children, some forms are treatable

Leukaemia Types

• Acute Leukaemia progression is FAST, including Blast Cells
• Chronic Leukaemia progression is SLOW, including More Mature Cells
• Can be Lymphocytic or Myelogenous
• Presents with a Primary Bone Marrow and Short History of Bruising, Bleeding/Infection, sometimes Skin Rash

Lymphadenopathy

• Note any Lymphadenopathy, Gingival Hypertrophy, and Recurrent Nosebleeds, a
• Check for primary bone marrow, disorders of acute Leukaemias, fever, chills, weakness, fatigue, unintentional weight Loss, enlarged lymph Nodes, enlarged Liver, or Spleen

Acute Leukaemia (Treatment)

• Treatment is subtype-dependent, Many patients in clinical trials, bone marrow transplantation may be performed
• Survival rates differ across leukemia types and treatment options, and with elderly patients prognoses significantly worsen
• Can lead to pancytopenia (all blood types decreased)

Myeloma

• Myeloma can lead to Marrow Infiltration and Osteolytic Deposits and cause many radiolucencies, shown up on radiographs.

Myelodysplastic Syndrome (MDS)

• Immature Cells are in the Bone Marrow and
• Can leads to be sometimes discovered on blood tests while asymptomatic, or, presenting with symptoms of anaemia Controlled with stem cell transplants, Chemo, and Replacement therapies

Malignancies of Head & Neck

• Malignancie lead to, Neoplastic proliferation of bone marrow white blood stem cells associated with specific gene.

Multiple Myeloma

• Nearly all patients take bisphosphonates with a median age of around 67. Anemia, bone pain, infection or Constipation, and stomach Upsets are common signs of this disease, or a low iron count
• Will show a Normochromic Normocytic Anaemia, raised ESR indicates indications of disease but not what type.

Lymphoma

• Lymphoma a malignant transformation of Lymphocytes
• Lymphoma includes the cancers of the lymphatic system with non-Hodgkin lymphoma being a high and lower grade
• Reed Sternberg Cells indicates the hallmark, and will be shown in in enlargements of lymph nodes in the neck and Axillae

Coagulation Disorders

• Haemophilia A - Factor VIII Replacement is severity dependent and treatment includes: DDAVP and ANTI-FIBRINOLYTICS, always consult with haematologist.
• Factor VIII deficiency, X-linked inheritance must liaise with haematologist

Haemophilia B

• Factor IX deficiency, range of severity, also consult with haematologist.
• DDAVP Ineffective often presents

Von Willebrand Disease (VWD)

• Inherited and always consult with hematologist.
• VWF is a protein seen in platelets, affects platelet Adhesiveness and Migration.
• Can range in severity; subtype 1, 2, and 3 depending on if VWF is dysfunctional and the amount
• Depends on planned Procedure, Previous Experience, and Sub-Type, may also include DDAVP, and note any ANTIFIBRINOLYTIC DRUGS

Factor V Leiden Thrombophilia

• Due to ABNORMAL FACTOR V, when deficient.

Bleeding Disorders (Principles)

• Always consider Local Haemostatic Measures, and Suturing Sockets,
• Consider Tranexamic acid, as well, and Mouthwash very gently
• Need to avoid NSAIDs and address Decreased Vit K.

Haemophilia

• Close Liaison with HAEMOPHILIA CENTRE, and do DDAVP Infusion for mild haemophilia and Do Factor VIII/IX replacement when the signs are moderate/Severe conditions.
• Utilize Atraumatic Techniques for local Haemostatic Measures and tranexamic acid.

Management of VWD Patients

• Manage with tranexamic acid, but also always treat for inherent defects.

Platelet Abnormality

• WARFARIN needs to be 4 OR LESS and monitor HEPARIN using aptt for 6 hours if possible, can use,
• LOW Molecular Weight Heparins use oral Direct Oral Anticoagulants (DOACs) for cases where there may be simple or complex extractions where there is need, Rivaroxaban for use for cases with Thrombin Inhibition, Not reflected in INR, and use rivaroxaban!

Different Drugs and Their Effects

• The process of coagulation is extremely complicated making different approaches very needed in a variety of ways
• Antiplatelet drugs will work for the effects of the initial platlets
• Anti-Coaguilants focus on the activity on platlets, and activity in a certain cascade

INR Levels

• Should be at 4 or less for procedures, otherwise, the patient can bleed
• Can influence or decrease, note diet habits so you know where to affect certain parts and what could go wrong.

NOAC use

• Use when cannot use Warfarin, those being Dabigatran or Rivarobaxan, have a low risk of bleeding or complications- with a general advice , note what needs to be treated through it. Treat to when the procedure can be tested post or not.

Managing a Patient with Medication

• Note patient medication as necessary to treat or be wary when treatment would have to be stopped to reduce the risk of bleeding during an operation procedure
• As that, check for if they need advice or a consult, or treat without for oral risks.
• Advise about what may need to be adjusted after.

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