A 14 YEAR OLD BOY WITH SPLENOMEGALY,PANCYTOPENIA
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A 14 yr old male came to the opd with the chief complaints of swelling in the left side of the abdomen since the past 15 days along with cold and cough since the past 10 days.
HOPI:
patient was apparently well 15 days back ,then he developed tightness,pain in the abdomen which was insidious in onset,dragging type of pain in the left hypochondraic ,lumbar region,tender on palpation
he had cough 10 days back ,productive with sputum not blood tinged
patient is born out of grade 4 consanguinous marriage ,birth order is 4 .
He has 2 elder brothers and one elder sister - who expired at 5 years of age .
His sister was the first child and she was assymoptomatic till 2 years of age ,then she had shortness of breath and was rushed to hospital,where here condition detiorated and got admitted .They were told that she had splenomegaly and Her blood counts were decreasing .She was given multiple blood transfusions every 25 days for one year inspite of that she remained anemic ,she also underwent bone marrow biopsy twice .
According to parents she was given steriods also for one year before death .she never had jaundice or recurrent infections.
.Elder brother is 19 year old and second brother is -16 years old .
Second one had history of fever ,white coloured loose stools at the age of 5 years ,for which they went to nalgonda hospital .He was given some medications and it got resolved .But he was said to have anemia ,and he recovered according to parents with some medications.There was no jaundice and no history of blood transfusion.
Pedigree chart :
Patient history :
He is the youngest of all .
He was born after full term normal vaginal delivery with 4kgs birth weight ,cried immediately after birth .
He had jaundice at birth ,that resolved spontaneously .Physiological jaundice.
He is Immunised as per schedule .
At 8 months of age patient had history of altered bowel habits - one week of loose stools and one week of normal consistency stools for few days later he was passing loose stools every day for 2-3 months ,during which he became very cachetic .
He got admitted in a hospital ,in the discharge summary - they mentioned as suspected case of celiac disease ,chronic diarrhea ,chronic malabsorption .
During the hospital stay he was given Iv antibiotic and 2FFP transfusions .He recovered in 3 months according to parents .
On further asking parents ,they told he used to have recurrent colds ,cough and fever .He used inhalers for 1 year during winters in childhood . Though he wasn't admitted at any point , and there was no history of pnuemonia.
At 9 years of age - parents noticed neck swelling and they took him to ENT doctor ,later referred to endocrinologist ,where he was diagnosed with hypothyroidism - intial TSH was -150 ,he was started with 150 mcg of thyronorm,later increased to 180 mcg .
patient also gives history of chronic itching over hands and foot since the age of 9 years
.At 12 years of age patient had chicken pox ,resolved over 10 days
9 months ago
at age of 13 years - he came with history of yellowish discoloration of eyes since 9 days .
One episode of bilious vomiting .
He also had complaints of yellow colored urine
No h/o pale colored stools .
No h/o fever ,pain abdomen ,loose stools, cold , cough .
no history of small joint pains .
He was treated by a pediatrician for jaundice ,but as the bilirubin is increasing they referred here for further management
past history: No h/ o DM,HTN ,CAD
epilepsy,TB,
personal History:
DIET: mixed
APPETITE: good
BOWEL AND BLADDER: regular
SLEEP: adequate
ADDICTIONS: none
family history : there is a history of death of patient's sibling in her early years of life,she received blood transfusion for many times ,she had splenomegaly
General physical examination: patient is conscious ,coherent,cooperative well oriented to time ,place and person
He is thin built and moderately nourished
Height -156 cm
Weight - 32kgs
pallor + ,icterus +
clubbing,cyanosis,lymphadenopathy - ve
systemic examination:
Per abdomen - moderate splenomegaly, tender,not soft .
umblicus central and normal
shape of abdomen : scaphoid
no scars and sinuses, presence of rashes
CNS : no focal neurological deficits
CVS : s1 and s2 heart sounds heard,no murmurs
RS: NVBS,BAE +
investigations -
There is significant drop in hemoglobin from 8.9 to recent HB -5,leucopenia , thrombocytopenia.
With raised bilirubin - both direct and indirect , normal enzymes .
Urine for bile salts and bile pigments + ve
HB electrophoresis - normal .
Serology for HiV ,HBSag , Hcv was negative
Dengue , Malaria ,widal was negative
At present:
He noticed a swelling in the left side of the abdomen since the past 15 days along with cold and cough since the past 10 days.
Pain is at left side of the abdomen and is of dragging type, non radiating.
General examination showed thin built and moderately nourished with massive splenomegaly
Assessment :
? Spectrum of Autoimmune diseases -
Thyroditis ,? AIHA
Chronic itching of both hands and limbs
? CVID
Plan - repeat CBP with peripheral smear ,LDH ,retic count ,coombs test .
Other routine investigations
Dermatology opinion .
Investigations done in our hospital :
Hemogram showed -
Normocytic normochromic anemia with HB 5.1
WBC count -1700 ,neutrophils -39% ANC-663 ,Esoniphils -8% .
Platelet count -1.5 lakh
With few microcytes ,tear drop cells ,pencil forms.
Coombs test
AMC bed 3 14yr old male
Admit on 26/08/22
Day 3
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/80mmhg
PR: 89
RR: 24
Temp: 99.2f
GRBS: 83mg/dl -8am
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? AIHA, URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly
Xray 28/08/22
Admit on 26/08/22
Day 4
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/70mmhg
PR: 84
RR: 22
Temp: 99.2f
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? AIHA, URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly.
AMC bed 3 14yr old male
Admit on 26/08/22
Day 5
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/80mmhg
PR: 82
RR: 22
Temp: 97.9f
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? AIHA, URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly
AMC bed 3 14yr old male
Admit on 26/08/22
Day 6
S: no fresh complaints
O: pt is conscious, coherent,cooperative.
BP: 110/80mmhg
PR: 80
RR: 20
Temp: 97.9f
Spo2: 98%
RS: BAE+ clear
CVS: s1,s2 no added sounds
P/A: not tender
CNS:NAD
A: pancytopenia with massive splenomegaly
Thyroditis ,? AIHA, URTI
P: plan of treatment
1.Inj. Neomol 1gm IV SOS
2.Tab dolo 650 mg PO SOS
3.Tab. Levocetrizine 5mg PO SOS
4.Syrup ascoril 10 ml PO BD
5.tab. thyronorm 200mcg, PO OD
6.inj. optineruron 1 amp
7. BP temp charting 6th hourly
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