Multiple myeloma

It is a lymphoproliferative disorder associated with monoclonal gammopathy that can present as abnormal bone fractures,renal failure,blood disorders,hypercalcemia etc.

One of the characteristic feature is Bence Jones proteins.

Diagnosis is established by a bone marrow examination which will show presence of plasma cells >30%in the marrow.

Diagnostic criteria:

• Clone bone marrow plasma cells >10%
• Presence of serum/urine monoclonal protein
• Evidence of end organ damage like hypercalcemia (Sr calcium >11.5mg/dl),Renal insufficiency(Sr creatinine >1.73mmol/L),Anemia (Hb <10g/dl),Bone lesions

Treatment:

    The main stay of treatment includes Corticosteroids in combination with Chemotheraphy (thalidomide,bortezomib,melphalan)

Allergies

It is a condition in which there is an increased immunological response by the body to a substance.There is a wide spectrum of manifestation of symptoms ranging from simple itching to life threatening anaphylaxis.
Manifestations may be itching,urticaria,eosinophilia,angioedema,anaphylaxis..These reactions are mediated through IgE mediated. For it to occur the body must have been initially sensitised with particular allergen.It is an hypersensitivity reaction.
Types of Hypersensitivity :

1. Type I-immediate hypersensitivity
2. Type II-Antibody mediated
3. Type III-Immune complex mediated
4. Type IV- T-cell mediate

It comes under type I hypersensitivity.It is mediated by IgE  secreted by B-cell, IgE binds to the mast cells causing the release of mediators for the reaction on exposure to the same antigen again.This whole process occurs within minutes,hence the name.

Mediators:

• Histamines
• Leukotrienes
• PAF
• Prostaglandins

Management:

• The main stay of management is the complete avoidance of the stimuli.
• Desensitisation of the allergen with serial exposure of the patient with varied subclinical concentrations of the antigen.
• Most of the cases respond well to symptomatic management with anti histaminics and time
• Active management is  life saving in cases of angioedema and anaphylaxis.In these cases epinephrine is the life saving drug, along with intravenous fluids.

Blood pressure

It is the common complaint nowadays in most of the patients.
Normal values varies with age.In cases of children mostly it  is not given much of importance. In children it varies with height.
In case of adults the normal is systolic <130mm Hg, diastolic <90 mm Hg.But we cant diagnose a person as an hypertensive based on single reading.Atleast 3 readings on 3 consecutive days most be higher than mentioned.
Hypertension is one of the most common disease and one of the most common cause of death world wide.
It is an end organ disease. It affects most of the organs among which most important is brain,kidney,retina(part of eye). It is also a main risk factor for atherosclerosis which in turn causes a lot of diseases in the body,most commonly heart attack(myocardial infarction).Hence screening is essential.
The age of onset is decreasing due to the unhealthy life style.
Causes:

1. Essential hypertension( unknown cause)
2. Renal failure
3. hyperthyroidism,hyperparathyroidism,cushing'syndrome etc
4. Drugs like corticosteroids,NSAIDs,Oral contraceptive pills

Risk factors:

1. Hereditary
2. Diabetes mellitus
3. Obesity
4. Unhealthy lifestyles

Iron deficiency anaemia- Diagnosis

Symptoms & Signs:

                -Certain clinical conditions carry an increased likelihood of the disease.,Pregnancy, adolescence, periods of rapid growth, and an intermittent history of blood loss of any kind should alert the clinician of the same. 

                 -A cardinal rule is in an adult male it is due to gastrointestinal blood loss until proven otherwise. 

                  -Signs depend on the severity and chronicity of the disease in addition to the usual signs—fatigue, pallor, and reduced exercise capacity.

                  -Cheilosis (fissures at the corners of the mouth) and koilonychia (spooning of the fingernails) are signs of advanced deficiency.

                  -The diagnosis is typically based on laboratory results.

Laboratory diagnosis:

                     -The serum iron level represents the amount of circulating iron bound to transferrin. The TIBC (Total iron binding capacity)is an indirect measure of the circulating transferrin. The normal range for the serum iron is 50–150 micro gm/dL; the normal range for TIBC is 300–360micro gm/dL.
                      -In the disease- serum iron is < 30micro gm/ dL &
                                                                     -   TIBC is >360 micro gm/dL
                      -Transferrin saturation, which is normally 25–50%, is obtained by the following formula: serum iron x 100 ÷ TIBC. the disease is associated with saturation levels below 18%.
                      -Adult males have serum ferritin values averaging about 100 micro gm/L, while adult females have levels averaging 30micro gm/L. As iron stores are depleted, the serum ferritin falls to <15 micro gm/L. Such levels are diagnostic of absent body iron stores.
                      -Peripheral smear will show hypochromic RBC & microcytosis

                    

Iron deficiency anaemia-Causes

It is the most common cause of decrease in hemoglobin world wide.

Causes:

        1.Increased demand for iron and/or hematopoiesis

                  -rapid growth in infancy or adolescence

                  -pregnancy

                  -erythropoietin therapy

        2.Increased iron loss

                  -chronic blood loss

                  -menses

                  -acute blood loss

                  -blood donation

                  -phlebotomy as treatment for polycythemia vera

        3.Decreased iron intake or absorption

                  -inadequate diet

                 -malabsorption from disease (sprue, Crohn's disease)

                 -malabsorption from surgery (post-gastrectomy)

                 -acute or chronic inflammation

GERD(Gasro-Esophageal Reflex Disorder)

It is a condition caused due to gastric contents into the esophagus and proximal structures.

Clinical Presentation:

• symptoms- heart burn and regurgitation
• Atypical chest pain
• Extraesophageal manifestations-cough,laryngitis,asthma and  dental erosions. Also  there may be sinusitis,pulmonary fibrosis,pharyngitis and recurrent otitis media.
• Response to therapeutic trial of PPI can be diagnostic but a negative response does not exclude the disease

Investigation:

• Endoscopy to rule out other causes of such esophageal symptoms, like eosinophilic esophagitis,candidial esophagitis etc
• 24hrs esophageal pH monitoring (investigation of choice)
• Esophageal manometry to identify motos process contributing to refractory symptoms

Treatment:

•  Antacids,histamine-2 receptor antagonists,PPI may relieve symptoms in mild and intermittent conditions
• PPI(Proton Pump inhibitors) are more effective than other treatment modalities.
• In conditions not responding to medical treatment,they are surgically managed by Nissen's Fundoplication

Oligoclonal Bands

Electrophoresis of serum and CSF separates protein components by size and charge.They may be present in both serum and CSF.When present in CSF alone, it indicates intrathecal synthesis of immunogloblins.This pattern is seen in 95% cases of established multiple sclerosis, it can also occur in chronic meningitis,neurosarcoid,neuromeningitis and SSPE 

Peripheral neuropathy

These patients will complaint of numbness in hands and feet that progresses proximally in a distribution classically termed as 'glove &stocking'
Causes:

1. Idiopathic (most common)
2. Diabetes mellitus
3. Vit B12,E deficiencies
4. Carcinoma
5. Drugs eg : isoniazid,dapsone,vinca alkaloids,cisplatin, gold, metronidazole
6. Paraproteinemias eg : myeloma
7. Rare causes like amyloidosis,GBS,sarcoidosis etc

Investigation of choice:

             Nerve conduction studies

Heinz bodies

These are red cell inclusion bodies made of insoluble denatured proteins.They are seen when the red cells are stained with methyl violet stain and supravital staining.


Sample to be taken: Blood mixed with EDTA.

Significance:They are are seen close to the RBC membrane.they are removed in the spleen and so their presence is frequent in splenectomy.
Causes:
        1.Oxidative hemolysis
                          -chlorates,phenacetin etc
                          -G6PD deficiency and other enzymopathies
        2.Unstable hemoglobins