1. Mahatma Gandhi Medical
College And Research Institute
Puducherry
DEPARTMENT OF INTERNAL MEDICINE
CASE PRESENTATION
HEAD OF DEPARTMENT : Prof. Dr. Jaya Singh
NAME OF GUIDE : Prof. Dr. Lokesh
NAME OF PRESENTOR: Thannushree. A. B
2. CASE SCENARIO
A 54 yr old middle aged female women Mrs. X who
belongs to the lower socio-economic class according
to Kuppuswamy scale came to our hospital with
CHIEF COMLAINTS OF
Fever - 10 days
Easy fatigability - 7 days
Breathlessness - 7 days
Abdominal pain - 7 days
3. HISTORY OF PRESENTING ILLNESS
My patient was apparently normal 10 days back
from the day of examination when she developed
FEVER
• 10 days in duration high grade ,
• Intermittent in nature ,
• Relieved on medications ,
• Associated with chills and rigor .
• Not associated with intense sweating or
evening rise in temperature .
4. HISTORY OF PRESENTING ILLNESS
She also presented with history of
EASY FATIGUABILITY and BREATHLESSNESS – 7 days
• Insidious in onset
• Gradually progressed from CLASS II to CLASS III grade
according to NYHA Grades of Dyspnoea
• Presently at class III Grade of Dyspnoea
• Associated with bilateral pedal edema and
palpitations for 5 days lately after the onset of
breathlessness
5. HISTORY OF PRESENTING ILLNESS
History of abdominal pain – 7 days
– Continuous
– Diffuse dragging pain
– Non radiating ,
6. NEGATIVE HISTORY
No history of
– Cough with rusty sputum,
– Orthopnoea , PND
– Burning micturation
– Syncope , chest pain
– Nausea ,vomiting , altered mental state, seizures
– Passage of blood or black colored tarry stools
– Passage of worms in stools or perianal pruritis or
generalized pruritis.
– No history of jaundice in the past
7. PAST HISTORY
• No history of similar complaints in the past
• Known case of HYPOTHYROIDISM - 20 years
- on replacement therapy with L-THYROXINE 75 mcg OD
- compliant with the medications.
• Known case of TYPE II DIABETES MELLITUS - 7 months
- oral hypoglycemic drugs(METFORMIN&GLIMIPRIDE)
- compliant with the medications
• Not a known case of Hypertension, Bronchial asthma,
IHD , or exposure to known case of pulmonary TB
8. MENSTRUAL AND PAST OBSTETRIC HISTORY
• Age of menarche – 14 years
• Irregular menstrual cycles of 5/45 with bleeding
period of 5 days
• No history of foul smelling discharge or inter
menstrual bleed or whitish discharge or passage of
blood clots
• Married since 34 years with an obstetric score of
G3P3 ,normal vaginal delivery with no complications.
• No history of miscarriages
9. PERSONAL HISTORY AND FAMILY HISTORY
• On mixed diet
• No disturbances in sleep and appetite
• Adequate sleep
• No disturbances in bowel habits
• No addictions
• No history of similar complaints in the family
10. TREATMENT HISTORY
• She Is on medications for , HYPOTHYROIDISM and
TYPE II DIABETES MELLITUS for 20 yrs and 7
months respectively
• Compliant with her medications
• NO History of BLOOD TRANSFUSIONS
• No history of adverse drug reactions
• No history of allergy to any drug
• No history of alternate medicine intake
• No history of treatment for malaria
12. GENERAL PHYSICAL EXAMINATION
• Patient was conscious , oriented to time, place and
person ,co-operative, moderately built and
moderately nourished
Pallor – present
Icterus - present
Clubbing – absent
Cyanosis – absent
No significant lymphadenopathy , no Virchow’s node
Bilateral pitting pedal edema
JVP elevated by 8 cms of H2O from the sternal angle
14. VITALS
PULSE
• RATE: 100/ min
• RHYTHM: Regular
• VOLUME: High volume Bounding pulse
• CHARACTER: No specific character
• CONDITION OF VESSEL WALL: Normal
• DELAY: No Radio-radial or Radio- femoral delay
• PERIPHERAL PULSES: All peripheral pulses felt
• NO CAROTID BRUITS
TACHYCARDIA
15. VITALS
BLOOD PRESSURE:
RESPIRATORY RATE:
-23/min
TEMPERATURE:
101o F – Febrile
RIGHT LEFT
UPPER LIMB 130/80 120/80
LOWER LIMB 140/80 140/80
TACHYPNOEA
16. SYSTEMIC EXAMINATION
CARDIOVASCULAR SYSTEM EXAMINATION:
• On auscultation ,
- S1 & S2 normally heard
- Gallop rhythm present
- Ejection Systolic murmur at the base of the heart
(pulmonary area)
ABDOMINAL SYSTEM EXAMINATION:
• On palpation,
- Tender hepatomegaly of 16 cms span in the right
hypochondrial region
- Soft spleenomegaly palpable for 6 cms in the left
hypochondrial region.
17. OTHER SYSTEM EXAMINATION
RESPIRATORY SYSTEM:
Normal Vesicular Breath Sounds
No added sounds
CENTRAL NERVOUS SYSTEM:
- Optic fundus examination was normal
- Deep tendon reflexes are intact
- Romberg's Sign was Negative
- No evidence of Peripheral Neuropathy and
Posterior Column involvement
19. INVESTIGATIONS
HAEMATOLOGICAL INVESTIGATIONS:
• Hemoglobin : 3.8g/dl
• RBC:1.11million / cu mm
• TLC : 22,100 / cu mm
• DLC
- Neutrophils:63%
- Eosinophils:3%
- Lymphocytes: 30%
- Monocytes: 2%
• Platelets: 1.76 lakhs / cu mm
• Serum electrolytes : normal
• There was interference with blood grouping and Rh
typing
ANEMIA
LEUCOCYTOSIS
20. PERIPHERAL SMEAR EXAMINATION:
Shows evidence of HEMOLYSIS
- Anisopoikilocytosis with Polychromasia
- Increased Nucleated RBC
- SPHEROCYTES present
- LEUKOCYTOSIS with normal distribution
- Platelets adequate
- NO HEMOPARASITES
RETICULOCYTOSIS
21. BIOCHEMICAL INVESTIGATIONS:
• Serum Electrolytes were normal
• Serum LDH : 4170 U/L
( Reference value: 140 – 280 U /L )
• Serum HAPTOGLOBULINS DECREASED
• Serum C3: 0.78 g/ L
( Reference value : 0.8 – 1.5 g /L)
• Serum C4 : 0.101 g/L
( Reference value : 0.16 – 0.38 g / L)
EVIDENCE OF HEMOLYSIS AND
COMPLEMENT ACTIVATION
INCREASED
DECREASED
DECREASED
22. LIVER FUNCTION TEST :
- Total protein : 7.8g/dl
- Albumin: 4.6 g/dl
- globulin: 3.2 g/dl
- A/G ratio : 1.4:1
- BILIRUBIN:
Total: 6.1 mg/dl
Direct: 0.5mg/dl
Indirect: 5.6 mg/dl
- AST , ALT, Alkaline phosphatase NORMAL
INDIRECT
HYPERBILIRUBINAEMIA
23. Renal function tests were normal
Thyroid function tests – Euthyroid State
Viral markers were NEGATIVE AND NON REACTIVE
ECG showed NORMAL SINUS RHYTYM with SINUS
TACHYCARDIA.
X ray chest was taken which showed no features of
cardiomegaly
Ultrasound abdomen:
- Suggestive of Hepatospleenomegaly
- No evidence of Portal Hypertension
- No Para-aortic Lymphadenopathy
25. CHEST ROENTGENOGRAM
-Chest X- Ray PA view.
-Trachea midline .
-No rotation – spino-clavicular
distance normal .
-No mediastinal shift .
-NO CARDIOMEGALY .
-Bilateral lung fiels normal
-Bony cage is normal .
- Fundus gas shadow on the left
below the diaphragm.
27. COOMB’S TEST: Positive
- DAT : Positive for Anti IgG
- IAT : Positive at 40 C and negative at 22oC &390C
ANTI NUCLEAR ANTIBODY: (CLIA method)
- Positive (4.0) {<1.5 Negative ; >1.5 Positive}
ANTI ds DNA : Positive
EVIDENCE OF WARM AUTOANTIBODIES
EVIDENCE OF COLD AUTOANTIBODIES
30. DIAGNOSIS
An HYPOTHYROID , TYPE II DIABETIC middle aged women with
SLE presenting as
“AUTOIMMUNE MIXED HAEMOLYTIC ANEMIA”
- History suggestive of Anemia
- Clinical examination reveals Pallor and Icterus
- Reticulocytosis , spherocytosis and schistocytes
- Increased serum LDH, decreased complement levels.
- Indirect Hyperbilirubinemia
- Coomb’s test positive
- DAT and IAT suggestive of mixed type of autoimmune
hemolytic anemia
- ANA , ds DNA were positive with low complement levels.
.
31. MANAGEMENT
Patient was initiated on pulse
METHYL PREDNISOLONE 1g per day for 3 days
Switched over to ORAL PREDNISOLONE 1mg/kg/day
By 48 hours, patient’s hemoglobin increased to
4.8g/dl with slow regression of
hepatospleenomegaly
32. FOLLOW UP
• The patient was on follow up for 3 weeks and
Hemoglobin improved from 3.9 to 7.8 g/dL
• No evidence of hemolysis on repeat Peripheral Smear
• No blood transfusion was done during the entire
course of treatment
• Diabetes was controlled with insulin in view of patient
on steroid therapy
• Patient remained normotensive during the course of
follow up and remained Euthyroid
• Patient remained weight neutral during the course of
therapy
33. TAKE HOME MESSAGE
• Autoimmune Hemolytic Anemias are usually characterized by
presence of warm or cold auto antibodies to RBC’s.
• Here presence of both warm and cold auto antibodies makes
it a case of Auto Immune Mixed Hemolytic Anemia.
• The patient’s serum was reactive for ANA , ds DNA , and low
C3 which points towards the immunological criteria for SLE .
• Hence our diagnosis is AUTOIMMUNE MIXED HEMOLYTIC
ANEMIA with connective tissue disorder SLE .
• AIHA occurs in less than 5% of patients with SLE.
• Mixed AIHA occurs in less than 10% of patients with AIHA.
• Hence, a case of MIXED AUTOIMMUNE HEMOLYTIC ANEMIA
as the hematological manifestation of SLE is a very rare entity.
34. Treatment options
– Cytotoxic drugs like cyclophosphamide,
chlorambucil and azathioprine
– Patient who is not responding to
Immunosuppressive therapy can be treated with
RITUXIMAB which is a Monoclonal IgG 1
antibodies which are directed against CD20
antigen which is useful in the therapy of Auto
immune hemolytic anemia .
– Plasmapheresis / IV immunoglobulin
– splenectomy
35. REFERENCES
1. Harrison’s Principles of Internal Medicine 19th edition .
2. Davidson’s Principles of Medicine 22 nd edition .
3. William’s Textbook of Hematology 8th edition.
4. Robbin’s Textbook of Pathology 8th edition.
5. Lange Basic and Clinical Pharmacology 13th edition.