Smouldering Multiple Myeloma

Disease class: Hypergammaglobulinaemia
Disease class: Paraproteinaemias
Disease class: Precancerous conditions

This refers to a plasma cell dyscrasia, in which abnormal excessive levels of gamma globulins are detected in the blood. There are no symptoms, but the patient has an increased risk of progression to multiple myeloma.

Monoclonal gammopathies

• Monoclonal Gammopathy of Undetermined Significance 
• Smouldering multiple myeloma
• Multiple myeloma

Heavy chain disease

Disease class: Lymphoproliferative disorders
Disease class: Paraproteinaemias

Methaemoglobinaemia

Disease class: Haematologic diseases

Sulfhaemoglobinaemia

Disease class: Haematologic diseases

Immunoglobulin light-chain amyloidosis

Disease class: Amyloidosis

Disease class: Paraproteinaemias  

Haemoglobin C disease

Disease class: Haemoglobinopathies

Hydrops fetalis

Also known as

• Foetal hydrops

 

Causes

• Foetal erythroblastosis
• alpha-Thalassaemia

delta-Thalassemia

Disease class: Thalassaemia

Cryoglobulinaemia

Disease class: Haemostatic disorders

Disease class: Paraproteinaemias

Monoclonal Gammopathy of Undetermined Significance

Disease class: Hypergammaglobulinaemia
Disease class: Paraproteinaemias

Also known as

• MGUS

This refers to a plasma cell dyscrasia, in which abnormal excessive levels of gamma globulins are detected in the blood. There are no symptoms, but the patient has an increased risk of progression to malignancies such as multiple myeloma. One study found that the risk of progression to malignancy was 11% (6.5 times higher than the general population).

 

Monoclonal gammopathies

• Monoclonal Gammopathy of Undetermined Significance  
• Smouldering multiple myeloma
• Multiple myeloma

Hypergammaglobulinaemia

Disease class: Blood protein disorders
Disease class: Immunoproliferative disorders 

Types

• Mevalonate Kinase Deficiency 
• Monoclonal Gammopathy of Undetermined Significance 
• Smouldering multiple myeloma

Paraproteinaemias

Disease class: Blood protein disorders
Disease class: Immunoproliferative disorders 

Types

• Cryoglobulinaemia
• Heavy chain disease 
• Immunoglobulin light-chain amyloidosis
• Monoclonal Gammopathy of Undetermined Significance 
• Multiple myeloma
• POEMS syndrome
• Smouldering multiple myeloma
• Waldenstrom macroglobulinaemia

Shwartzman phenomenon

Disease class: Haemostatic disorders

Cavernous haemangioma

Disease class: Haemostatic disorders

[Haematology] Introduction

Haematologists specialise in diseases of the blood. This includes the specialised blood cells and their proteins, particularly haemoglobin.
The spleen and bone marrow are closely associated with haematological diseases.
There is significant overlap with the fields of oncology and immunology.
This is a pathology discipline.  

 

Subspecialties

• Haemato-oncology
  

 

Etymology

Ancient Greek αἷμα (haîma) = blood
Ancient Greek λογία (logia) = study

 

Responsibilities

• Providing care to patients from their first blood test through to diagnosis and treatment.
• Blood transfusion.
• Marrow stem cell transplantation.
• Genetic testing.
• Guiding decisions for chemotherapy and other treatments.
• Giving advice to GPs and other hospital specialists.

 

Investigations

 

Relevant problems:

• Anaemia  
• Thrombocytopenia
• Leukopenia
• Pancytopenia
• Coagulopathies 
• Haemophilias
• Malignancies of the blood and bone marrow 
• Paraproteinaemia   

 

Haemic and lymphatic diseases

 

Relevant diseases:

• Leukaemia 
• Hodgkin lymphoma
• Non-Hodgkin lymphoma 
• Myeloma  
• Sickle cell disease
• Thalassemia
• Essential thrombocythaemia (ET) 
• Polycythaemia vera (PV)
• Myelofibrosis (MF) 
• Myelodysplastic syndrome (MDS)
• Porphyria  
• Plasmacytoma 
• Monoclonal gammopathy of unknown significance (MGUS)  
• Amyloidosis
• AL amyloidosis

Acute radiation syndrome

Disease class: Radiation injuries

Also known as

• ARS

 

Symptoms and signs

• Anorexia
• Nausea
• Vomiting
• Diarrhoea
• Haematemesis
• Pain
• Confusion
• Coma
• Haemoptysis
• Dyspnoea
• Erythema
• Oedema
• Alopecia
• Nail loss
• Tooth loss
• Blistering
• Desquamation
• Pain
• Haemorrhage
• Bruising
• Weakness
• Numbness

 

Pathological features

• Gastrointestinal dysfunction
• Gastroenteritis
• Gingivitis
• Necrosis
• Neurological dysfunction
• Neuritis
• Cerebral oedema
• Confusion
• Coma
• Pulmonary dysfunction
• Pneumonitis
• Pulmonary oedema
• Cardiovascular dysfunction
• Myocarditis
• Heart block
• Vasculitis
• Haemorrhage
• Mucocutaneous dysfunction
• Dermatitis
• Tissue necrosis
• Dental caries
• Haematological dysfunction
• Sepsis
• Thrombocytopenia
• Neutropenia
• Lymphopenia
• Aplastic anaemia
• Musculoskeletal dysfunction
• Myositis
• Osteonecrosis
• Multiple organ failure

 

Aetiology

• ARS follows exposure to an ionising radiation dose of more than 1 Sievert.
• A dose of 4-5 Sieverts or more, absorbed within a short time period, causes death in 50% of patients within 30 days.

 

Pathophysiology

• High energy ionising radiation causes damage to all exposed biological structures.
• The energy of the radiation can result in tissue damage similar to thermal or electrical burns. A sufficiently high dose will vaporise living tissue.
• The most radiation-sensitive structures within cells are the genetic materials. DNA and RNA are easily damaged or destroyed, leaving the cells incapable of functional protein synthesis.
• Successful mitosis cannot occur so destroyed cells are not replaced.
• The cell cannot repair physical damage.
• Many cells undergo apoptosis (programmed cell death).
• Tissues start to break apart.

 

Management

• Iodine tablets can saturate the biosynthesis pathways inside the thyroid gland. This minimises the uptake of iodine radioisotopes from the environment and reduces the risk of thyroid cancer.
• Supportive care is vital to minimise disability and death in the short term.
• In the long term, these patients should be monitored for signs of malignancy and bone marrow dysfunction.

 

History of radiation injuries

Immunologic deficiency syndromes

Disease class: Immune system diseases

Types

• Agammaglobulinaemia
• Ataxia telangiectasia
• Common variable immunodeficiency
• Dysgammaglobulinaemia
• HIV infections
• Deltaretrovirus infections
• Leukocyte-adhesion deficiency syndrome
• Lymphopoenia
• Phagocyte bactericidal dysfunction
• Severe combined immunodeficiency 

This class includes all diseases caused by underactivity of the components of the immune system. 

Patients with these diseases have a significantly increased probability of severe or life-threatening infections. 

Immunoproliferative disorders

Disease class: Immune system diseases

Types

• Hypergammaglobulinaemia 
• Lymphoproliferative disorders 
• Paraproteinemias 

beta-Thalassaemia

Disease class: Thalassaemia

alpha-Thalassaemia

Disease class: Thalassaemia