ERPM PART B & C

JAN / FEB 2017

Second Edition - Modified and Updated Version

LONG CASES
Anemia, Hemophilia, Rheumatology
Name
..................................................

Dr. Ali Akram

Registrar in Medicine
National Hospital of Sri Lanka
Colombo
0773855009
aliakram20@gmail.com

A. APPROACH TO AN ANEMIC PATIENT

Microcytic - SLIAT
Sideroblastic Anemia

Normocytic
Anemia of Chronic Disease

Macrocytic MY FAT RBC

Myelodysplastic syndrome

Lead Poisoning

Fetus (Pregnancy)

Iron Deficiency

Alcohol

Anemia of Chronic Disease

Thyroid (Hypothyroidism)

Thalassemia

Reticulocytosis (Hemolysis)
B12 and Folate Deficiency
Cirrhosis

Possible Presenting Complaints of An Anemic Patient

Decreased O2 Delivery
Exertional dyspnea
Dyspnea at rest
Fatigue
Palpitations
Confusion
Heart Failure
Arrythmia
Myocardial Infarction

Acute and Marked Bleeding

Hematemesis / Melena / Brisk PR
Bleeding / Vaginal Bleeding
+
Fatiguablity
Muscle cramps
Postural dizziness
Syncope
Persistant hypotension
Shock

A patient with with anemia can have I.

Isolated anemia - (above causes)
II.
Part of Pancytopenia
A.
Bone Marrow Failure
I.
Aplastic Anemia - Drugs, Chemicals, Radiation, Parvovirus
II.
Marrow Infiltration - Lymphoma, Leukemia, Granuloma (TB)
III.
Marrow Fibrosis - Myelofibrosis
IV.
Abnormal Differentiation - Myelodysplastic Syndrome
B.
C.
D.

Hypersplenism - (any cause of splenomegaly)

Systemic Lupus Erythematosus (SLE)
Paroxysmal Nocturnal Hemoglobinuria

History of Presenting Complaint

This depends on the particular presenting complaint given above.
Eg - Shortness of breath on moderate exertion
Patients with Thalassemia may rarely NOT have a proper presenting complaint, as
they would have been admitted for routine blood transfusion.

Dr. Ali Akram

Page 01

Systemic Review

Generalized well being - Hypothyroidism, TB, Non-specific in Anemia

Prolonged fever - TB, Opportunitic infections in any pancytopenia
Headache, visual disturbances (Non-specific anemic symptoms)
Recurrent sorethroat - Opportunitic infections in any pancytopenia
Dyspepsia, abdominal pain related to foods, melena, hematemesis
Loss of weight, loss of appetite, bleeding PR - GI Malignancies
Chronic diarrhea - GI TB, Inflammatory Bowel Diseases
Lower limb weakness and numbness - Vertebral metastasis and polyneuropathy
Back pain - Metastasis in malignancies
Skin rashes (photo-sensitive) - Autoimmune conditions like SLE
Bleeding manifestations - epistaxis, bleeding from gums - Pancytopenia
Menstrual history (Heavy Menstrual Bleeding)
A.
Duration
B.
Number of sanitary pads per day (fully soaked or half-soaked)
C.
Any need for double protection? (using two pads at once)
D.
Passing of any clots?

Past Medical History

History of
I.
T2DM
II.
CKD
III.
IHD
IV.
TB
V.
Bronchiectasis and COPD
VI.
Cirrhosis
History of any autoimmune rheumatoid diseases
History of Hypothyroidism
History of blood disorders

History of abdominal (gastric) surgeries

Drug History

Over the counter medicines - (Mainly for the aches and pains)
NSAIDS causes gastritis
Ayurvedic medicines - can cause cirrhosis
History of worm treatment

Family History

Family history of congenital blood related disorders

Family history of blood malignancies
Contact history of Tuberculosis

Social History

Page 02

Occupational exposure to chemicals

Smoking in pack years

Dr. Ali Akram

SAMPLE HISTORY, SUMMARY, PROBLEM LIST AND

INITIAL MANAGEMENT
Patient with Anemia

40 year old patient

Housewife
From Homagama

Admitted to ward one day before

She was apparently well before two weeks duration
Then she has developed gradual onset of worsening shortness of breath for two weeks
duration
Initially it was on streneous exertion
Now it comes with moderate exertion
There was no associated chest pain or tightness
She denies wheezing or cough
There was no orthopnea or PND
She also had associated fatigue and malaise also
There is also no abdominal or lower limb edema
But she doesnt have cold intolerance, constipation and hoarseness of voice
There is no history of fever
She denies any bleeding manifestations
She also doesnt have skin rashes or joint or back pains
Bowel and urinary habits are normal

She is a known patient with T2DM for 5 years

It was diagnosed incidentally by a GP when she had a fever
Patient takes medicine from pharmacies
She is currently on Metformin 500mg trice daily
There is no proper continuation of medications
She didnt undergo any end organ screening tests for diabetes mellitus
She doesnt have ischemic heart disease, hypertension or dyslipidemia

She didnt undergo any surgeries before and he is not allergic to foods or drugs

She is not taking any medicines including over the counter drugs and Ayurvedic medicines except for the Metformin
She has not taken the worm treatment in recent past

She is having regular menstrual cycles

It lasts for 4 to 5 days
There is no features of heavy menstrual bleeding

Her husband is a businessman

She has three children
Family income is adequate

Dr. Ali Akram

Page 03

Summary
40 year old known patient with T2DM for 05 years presented with worsening shortness of
breath on moderate exertion for two weeks duration. She also has non-specific symptoms of
fatigue and malaise.
I would like to examine the patient further.

Assume that you examined the patient and she is pale. No glossitis, koilonychia.
There is mild bilateral pitting ankle edema, but no ascites.
There is no organomegaly.

Problem List
1.

Acute Problems
A.
Exertional dyspnea of moderate exertion, probably due to anemia
B.
Bilateral lower limb edema - can be I.
Related - Probable CKD (T2DM induced)
II.
Unrelated - Cirrhosis, Heart Failure, Hypothyroidism

2.

Chronic Problems
A.
T2DM

Initial Management Plan

1.

This patients symptoms were probably due to anemia

2.

The cause for this anemia should be evaluated

3.

I will arrange the following investigations

A.
Full Blood Count - to see the Hemoglobin, MCV and RDW
B.
Blood Picture
I will arrange the further evaluation depends on the MCV

4.

If the patient is having low MCV - I will do the I.

Iron Studies
II.
Serum Ferritin
III.
Stool for occult blood

5.

If the patient is having normal MCV - I will do the I.

I will look the blood picture for abnormal red cells
II.
I will look for hemolysis - High LDH, Indirect Billirubin, Low Haptoglobin
III.
I will look for blood loss
IV.
I will look for Serum Creatinine

6.

If the patient is having high MCV - I will do the I.

Check the blood picture for hypersegmented red cells
II.
Liver Enzymes
III.
TSH / fT4

Page 04

Dr. Ali Akram

B. APPROACH TO A HEMOPHILIA PATIENT

A hemophilia patient is admitted, because of I.
Not related to hemophilia (Eg - Fever, Chest Pain)
II.
Related to hemophilia
A.
Bleeding (from impaired hemostasis)
B.
Sequale from bleeding - hemathrosis, hematoma development and pain
C.
Complications of factor infusion - Blood borne infections, Inhibitors
III.
Factor replacement
Disease Activity
Severe Hemophilia A
Moderate Hemophilia A
Mild Hemophilia A

- <1% factor activity

- 1 - 5% factor activitiy
- 5 - 40% factor activity

History of Presenting Complaint

This depends on the particular presenting complaint given above.

Systemic Enquiry

Fever - non-specific (think of infectons)

Headache, altered mentor - intra-cranial bleeding, which is rare in adults
Nose, gum, oral mucosal bleeding
Hematemesis, melena
Joint swelling - hemarthrosis
Muscle swelling - hematoma

Past Medical History

I.
II.
III.
IV.
V.
VI.

Hemophilia Period of diagnosis

Circumstance of diagnosis
Severity
Prophylactic Therapies (Factor VIII or FFP)
Vaccination for Hepatitis
Any complications A.
Chronic Hemophilic Arthropathy
B.
Infection from plasma transfusion
C.
Development of inhibitors

Family History

Family history of Hemophilia

Social History

Working and sports

Smocking (increased CVS morbidity risk)

Dr. Ali Akram

Page 05

C. APPROACH TO A PATIENT WITH RASH AND JOINT PAINS

Common Causes of Rash and Joint Pains
Simple viral infection leading to exanthem
Systemic Lupus Erythematosus
Dermatomyositis
Psoriatic arthritis
Acute Rheumatic Fever (Erythema Marginatum)
Drug induced (Hydralazine, Isonaizid)

Features of Systemic Lupus Erythematosus

Clinical
Malar Rash
Discoid Rash
Photosensitivity
Oral Ulcers
Arthritis (non-erosive)
Serositis (Pleuritis/Pericarditis)
Seizures / Psychosis

Bochemical
Persistant Proteinuria / Cellular Casts
Hemolytic Anemia
Leukopenia
Lymphopenia
Thrombocytopenia
Anti-DNA Antibodies
Anti-Sm Antibodies
Anti-Phospholipid Antibodies
ANA

A SLE patient can present as A.

First episode of clinical manifestations
B.
Relapse of SLE
C.
Pyrexia of Unknown Origin
D.
Complications of treatment - Prednisolone induced side effects
E.
Non-related illness (Eg - chest pain, dysuria)

History of Presenting Complaint

Depends on the presenting complaint

Systemic Review

Page 06

Prolonged fever
Headache, altered behaviour - Cerebral Lupus
Sorethroat - Infections
Chest pain (pleuritic) - Pericarditis, Pleuritis
Progressive shortness of breath - Shrinking Lung Syndrome
Oral ulcers - feature of SLE
Loss of weight, loss of appetite - feature of SLE, Infections
Hematuria, frothy urine and reduced urine output - Renal Lupus
Symmetrial small joint pain and stiffness Limb weakness and numbness and features of Raynauds phenomenon
Skin rashes (photo-sensitive), mainly over the face
Alopecia- feature of SLE
Activites of daily living

Dr. Ali Akram

Past Medical History

Systemic Lupus Erythematosus I.

Duration / First diagnosed?
II.
What was the initial presentation?
III.
How many times got admitted with worsening symptoms?
IV.
Were there any neurological involvements before?
V.
Was renal biopsy done?
VI.
Contraception?

Recurrent pregnancy losses (secondary APLS)

Drug History

Over the counter medicines Some drugs can cause SLE - Hydralazine, Isoniazid

Ayurvedic medicines

Family History

Family history of autoimmune rheumatoid diseases

Family history of recurrent pregnancy losses

Social History

Civil status and children

Whether family completed or not?
Family support
Patients knowledge on the condition
Nearest hospital and rehabilitation centre
Smoking (particularly in males)

Drug use during pregnancy in treatment of autoimmune rheumatoid diseases (Eg SLE and RA)
Paracetamol

the oral analgesic of choice

Oral NSAIDS and Selective

COX-2 Inhibitors

can be used after the implantation up until the last trimester if

symptoms justify their use

Corticosteroids

May be used to control disease flares (main maternal risks are

hypertension, glucose intolerance and osteoporosis)

DMARDS

May be used
Sulfasalzine, Hydroxychloroquine, Azathioprine or Ciclosporin
Must be avoided
Methotrexate, Leflunamide, Cyclophosphamide

Dr. Ali Akram

Page 07

SAMPLE HISTORY, SUMMARY, PROBLEM LIST AND

INITIAL MANAGEMENT
Patient with SLE

27 year old
University student
From Ragama

Admitted to ward one day before

She was apparently well before two days duration
While she was going on a bus, she had an episode of generalized tonic clonic seizure
It was witnessed by her brother who accompanied her
Initially the patient fell down on the bus
Then developed rigidity for one minute
During that time her eyes rolled up and there was tongue bite
The tonicity was followed up by generalized clonic movements which lasted for
around one to one and half minutes
After than she became drowsy which lasted for around 30 minutes
There was no urinary or bowel incontinence
She never had a seizure before
Then the patient was taken to the hospital where initial blood sugar was
112mg/dl
She didnt have any fever, headache, altered behaviour or significant ill health
currently
Regarding her systemic enquiry, she has noticed the following for the last one
month duration Increased hair fall
Generalized joint pains and aches, but no obvious swelling
Photosensitive rash, mainly in the face
Generalized malaise and fatigue
There were no oral ulcers, chest pain, shortness of breath, frothy urine

Her past medical history is unremarkable

She didnt undergo any surgeries before and he is not allergic to foods or drugs

She is not taking any medicines including over the counter drugs and Ayurvedic medicines

She is having regular menstrual cycles

It lasts for 4 to 5 days
There is no features of heavy menstrual bleeding

She is unmarried
There is no family history of epilepsy, autoimmune rheumatoid illnesses

Page 08

Dr. Ali Akram

Summary
27 year old previously well patient presented with an episode of generalized tonic clonic
seizure in the background one month history of alopecia, genealized malaise, photosensitive
facial rash and arthralgia.
Acutely she doesnt have any features of CNS infections or space occupying lesions.

Problem List
1.

Acute Problems
A.
Generalized tonic clonic seizure
B.
Alopecia, Photosensitive rash, arthralgia, and constitutional symptoms

Initial Management Plan

1.

This patient is having a generalized tonic clonic seizure. This can be due to I.
First episode of adult onset epilepsy
II.
Secondary seizures - CNS Infections, SOL, Electrolyte Imbalances, Hypoglycemia
III.
With the background history - Cerebral Lupus

2.

The cause for the seizure should be evaluated

3.

I will arrange the following investigations

A.
Full Blood Count
B.
Capillary blood sugar
C.
Serum Electrolytes including Calcium, Magnesium and Phosphate
D.
Electroencephalogram
E.
NCCT Brain (CECT Brain and MRI Brain)
F.
Lumbar Puncture
G.
ESR and CRP

4.

For the evaluation of the possible background SLE, I would like to arrange I.
ANA
II.
ds-DNA
III.
Complement Levels - C3 and C4
IV.
Urine Full Report - Active Sediments

5.

As this is the first episode of a fit, Im not going to start an antiepileptic drug in this
patient

6.

If the patient is confirmed of SLE - I will start

I.
Oral Hydrochloroquine
II.
Oral Prednisolone

7.

I will discuss with the patient and family members regarding her illness, marriage,
heritance, contraception and follow up

Dr. Ali Akram

Page 09

D. APPROACH TO A PATIENT WITH JOINT PAINS AND

SWELLING
Common Causes of Joint Pains and Swelling
Viral Infections
Rheumatoid Arhtiris
Osteoarthritis
SLE, Dermatomyositis, Sjogrens Syndrome
Psoriatic arthritis
Reactive Arthritis

Clinical Features of Rheumatoid Arthritis

Articular

Extra-Articular

Classic RA
Metacarpophalangeal joint
Proximal interphalangeal joint
Metartasophalangeal joints

Subcutaneous nodules
Tenosinovitis

Elbow joints
Shoulder joints
Ankle joints
Knee joints

Mononeuritis Multiplex

Morning Stiffness

Bronchiectasis, Pleural Effusion,

Lung fibrosis, Caplans Syndrome
Pericarditis, Raynauds Syndrome
Peripheral Neuropathy
Compression Neuropathies
Sicca Syndrome, Scleritis

Palindromic Rheumatism

Proteinuria and Nephrotic Syndrome

Monoarthritis

Feltys Syndrome
Anemia

A RA patient can present as A.

First episode of clinical manifestations
B.
Relapse of RA
C.
Extra-articular manifestations
D.
Complications of disease (Eg - septic arthritis) and treatment
E.
Non-related illness (Eg - chest pain, dysuria)

History of Presenting Complaint

Depends on the presenting complaint

Systemic Review

Page 10

Prolonged fever
Eye pain, red eye and gritty sensation
Chest pain (pleuritic)
Progressive shortness of breath
Loss of weight, loss of appetite, malaise
Frothy urine
Limb weakness and numbness, loss of function, morning stiffness duration

Dr. Ali Akram

Past Medical History

Rheumatoid Arthritis I.
Duration / First diagnosed?
II.
What was the initial presentation?
III.
How many times got admitted with worsening symptoms?
IV.
What are the treatments given? Any biologics?

Past history of Tuberculosis?

Any surgeries related to RA done? Carpal tunnel decompression?

Drug History

Over the counter medicines

Ayurvedic medicines

Family History

Family history of autoimmune rheumatoid diseases

Social History

Civil status and children

Family support
Patients knowledge on the condition
Nearest hospital and rehabilitation centre
Smoking (particularly in males)

Management Principles of Rheumatoid Arthritis

Establish the diagnosis clinically
Start early physiotherapy and occupational therapy
Start DMARD as soon as the diagnosis made
NSAIDS and steroids are used as bridging therapies to rapidly achieve
control of inflammation until DMARS are sufficiently effective

Non-Biological DMARDS

Biological DMARDS

Hydrochloroquine

Infliximab

Methotrexate

Rituximab

Sulfasalazine

Tocilizumab

Dr. Ali Akram

Page 11