15 year old female with Common variable Immunodeficiency syndrome with auto immune hemolytic anemia

A 15 yr old female came to casualty with chief complaints of

1- Yellowish discoloration of eyes since 5 days  

2- Vomitings since 3 days

HISTORY OF PRESENTING ILLNESS

Patient was apparently asymptomatic 5 days ago then she developed 

Yellowish discoloration of eyes it was associated with dark coloured urine

Vomiting since 3 days , 3 episodes - Non bilious, non projectile , food as content

Mosquito bites 2 days ago followed by rashes on B/L limbs

No history of fever, abdominal pain, cough, headache, constipation, diarrhea

PAST HISTORY

K/c/o CVID with AIHA admitted in February and got discharged 

In detail Previous history during her last admission and her previous medical conditions (since birth) can be found in the case report link attached below -

https://chandanavishwanatham19.blogspot.com/2021/03/17year-old-female-with-recurrent.html?m=1

After the discharge there was h/o fall in hemoglobin 

Previous h/o 4 blood transfusions done

In August- 2 pint PRBS transfusion done

After August PRBC ,Since discharge

Pt is on IVIG, Oral steroids every month

Started on TAB AZATHIOPRINE 50mg OD

In September - 1 pint PRBS transfusion done

In september during her follow up

She is presently on TAB WYSOLONE 10mg OD,TAB AZATHIOPRINE 50mg OD

But according to attendants this time her yellowish discoloration is more severe

K/C/O Bronchial Asthma since 10 years

Diagnosed at Age 3Y , initially used Syrup , tablets and In 2016 at our hospital Inhaler was administered as the symptoms were severe and stopped using it 1 year later since symptoms subsided (Unknown medication)

 

Tuberculosis diagnosed at Age 3Y - Used aTT medication for 6 months

Not a K/c/o DM, Hypertension , Epilepsy

Personal history:

Appetite -normal

Bowel movement- regular

Micturition - normal

No known allergies

No addictions

No significant family history

General examination

Pallor +

Icterus +

No cyanosis,clubbing, lymphadenopathy and edema

Temp-98.6F.

Pulse: 80bpm

Respiration: 20 cpm

BP - 100/60 mmHg

SpO2 - 99% at RA

CVS:S1,S2 heard

RS: BAE +,NVBS heard

Per Abdomen - soft,non tender ,

                   Moderate splenomegaly

CNS - NAD

Investigations

CLINICAL IMAGES

ICTERUS

                         

RASHES ON HER BOTH LIMBS

Provisional diagnosis

Common variable immunodeficiency syndrome with auto immune hemolytic anemia with acute hemolytic episode

Treatment

1) Tab. Predinisolone 20 mg po/OD

2) Tab.Azathioprine 50 mg po/OD

3) Tab.Doxycycline 50 mg po /BD

4) Tab.Folvite 5 mg po/OD

5) Tab Orofer -XT po/OD

Opthalmology Referral was done on 18/11/2021 i/v/o of diminished visual acuity

DAY 2

S- No fresh complaints

O-

Pt is c/c/c

Afebrile 

PR-70bpm

BP-100/60mmHg

CVS- S1, S2 heard

R.S - BAE+ 

P/A- soft,non tender, 

Moderate splenomegaly

CNS-Hmf intact ,no FN

A- ACUTE ON CHRONIC HEMOLYTIC ANEMIA

  2° to ? Viral Hepatitis

CVID with AIHA

P -

1-TAB PREDNISOLONE 20mg PO/OD

2-TAB DOXY 50mg PO/BD

3-TAB FOLVITE 5mg PO/OD

4- TAB OROFER XT PO/OD

5- TAB SHELCAL PO/OD

6- CAP BIO D3 PO/OD ( Weekly once)

7- BP/PR/TEMP CHARTING 6 hrly

DAY 3

S- No fresh complaints

O-

Pt is c/c/c

Afebrile 

PR-67bpm

BP-90/60mmHg

CVS- S1, S2 heard

R.S - BAE+ 

P/A- soft,non tender, 

Moderate splenomegaly

CNS-Hmf intact ,no FN

A- ACUTE ON CHRONIC HEMOLYTIC ANEMIA

  2° to ? Viral Hepatitis

CVID with AIHA

P -

1-TAB PREDNISOLONE 20mg PO/OD

2-TAB DOXY 50mg PO/BD

3-TAB FOLVITE 5mg PO/OD

4- TAB OROFER XT PO/OD

5- TAB SHELCAL PO/OD

6- CAP BIO D3 PO/OD ( Weekly once)

7- SYP ARISTOZYME PO/BD

8- BP/PR/TEMP CHARTING 6 hrly