A 14 year old male with splenomegaly
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I have been given this case to solve in an attempt to understand the topic of " patient clinical data analysis" to develop my competency in reading and comprehending clinical data including history, clinical findings, investigations and come up with diagnosis and treatment plan. is an online e log book to discuss our patient de-identified health data shared after taking his / her / guardians signed informed consent. Here we discuss our individual patients problems through series of inputs from available global online community of experts with an aim to solve those patients clinical problem with collective current best evident based input.
A 14 yr old male from miryalguda, came to the opd with chief complaints of pain abdomen in left upper quadrant.
HOPI: PAatient was apparently asymptomatic performing his daily routine activities until 3 months ago , then he noticed a mass in left upper quadrant which is progressive in nature upto umbilicus which is painless , then on 24/10/22 back,he developed loss of appetite for 3 days and dull ache pain , which is insidious in onset in left upper quandrant , dragging type of pain continuous , aggravated on deep inspiration and partially relieved by medication associated with 2 episodes of vomiting which is non bilious, the mass now rapidly increased below umbilicus .
No history of fever (to rule out leishmania and malaria)
No history of weight loss, night sweats( to rule out malignancy)
no history of constipation and diarrhea.
PAST HISTORY:
NO history of blood transfusions
NO history of seizures
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 .
FAMILY HISTORY:
He is a 4th order child ,born out of grade 4 consanguinous marriage
His sister , who was the first child expired at 5 years due to complications of spleenomegaly.
PERSONAL HISTORY:
Diet : MIxed
sleep : adequate
appetite:normal
bowel and bladder regular
no habits
Daily activities: As he is a hostellite in gurukul, he wakes up at 4 0 clock ,freshen up does running and some exercises which are fatiguable for him than their friends , he takes thyroid tablet and freshen up, he eats breakfast , then he goes to school , he sits in first bench as he has a visual acuity of -4.2 and at 12 0 clock he eats lunch (except brinjal) , unitil 6 0 clock he has classes and then plays and have dinner and goes to sleep at 9pm .He have leg pains at night( restless leg syndrome?)
TREATMENT HISTORY:
he was on thyroxine 50 micrograms
GENERAL EXAMINATION:
On 30/11/22
patient is conscious , coherent and cooperative, thin built
height -156 cm
weight-32 kgs
conjuctival pallor +
icterus -
cyanosis -
clubbing-
lymphadenopathy cervicAl , can be palpated in 1B 0.5×0.5 cm firm non tender skin pinchable over the skin
Axillary , inguinal ( not given consent)
waldeyer ring
tonsils:
edema -
skin - chicken pox scars are seen
healed leg ulcers.
VITALS
BP: 110/70mm Hg in right brachial artery
pulse rate: 82 bpm regular, normal volume, normal character, normal peripheral pulses
temperature: afebrile
respiratory rate: 16cpm
SYSTEMIC EXAMINATION:
Abdomen : Soft , non tender
spleenomegaly: firm, non tender , smooth surface 2cm beyond the umbilicus, moving with respiration
liver span:
no free fluid.
CVS: apex beat in 5th intercoastal space 1cm medial yo mid clavicular line
s1 , s2 heard
RESPIRATORY SYSTEM: normal vesicular breath sounds all areas .no added sounds
CNS:
E4, V5,M6
No focal neurological deficits
PROVISIONAL DIAGNOSIS: SPLENOMEGALY( hypersplenism) 8cm below left coastal margin with anemia and generalized lymphadenopathy
DIFFERENTIAL DIAGNOSIS :
Gaucher disease, sarcoidosis, autoimmune hemolytic anemia with thyroditis, CVID, leukemia.
INVESTIGATIONS
peripheral smear:
on examination -
He was thin built .
Height -156 cm
Weight - 32kgs
Jaundice + ,pallor +
Per abdomen -soft, non tender with moderate splenomegaly
Outside 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
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.
Liver profile showing total bilirubin of 6.49, direct amounting to 0.52, with AST,ALT,ALP normal, total proteins of 5.5 and albumin of 3.8
Direct Coombs was positive
USG abdomen showing moderate splenomegaly.
Assessment :
? Spectrum of Autoimmune diseases -
Thyroditis ,? AIHA (warm body type) CVID
He was started on methylprednisalone 32mg IV for 5 days later on converted to oral dosage of 30mg and was tapered over 2 months.
And vitamin B12 1000mcg injections through the course of hospital and was continued for 6 months (standard b12 regimen ). And other symptomatic medications.
MY Questions on the day of presentation.
warm antibody mediated aiha causing extravascular hemolysis causing jaundice? factors favouring it:
urine bile -positive
direct coombs test - positive
leg ulcers as in heriditary spherocytosis (which is also EVH)https://pubmed.ncbi.nlm.nih.gov/14521562/.
spleenomegaly with thrombocytopenia and leukopenia( hypersplenism)
normocytic normochromic( outside investigations)
History of celiac?
Thyroiditis
*Factors not favouring it:
corrected retic count - shows hypoproliferative bone marrow, why?
No signs of hemolysis
why not gaucher?
INVESTIGATION FROM 11/11/21
He was referred to higher centre in view of portal hypertension secondary to ?NCPH/IPH, given the background of hypersplenism, massive splenomegaly with liver profile normal, portal vein doppler showing portal vein of diameter 1.6cms, with patent hepatic artery and vein, no collaterals and no ascites
He was also treated for tropical spleen with primaquine 30mg for 2 weeks, artemether and lumefantrine.
Bone marrow biopsy and lymph node biopsy showed reactive with no evidence of infiltrative etiology or malignancy in September.
X - Ray:
CT SCAN:
Halo sign in lung fields
BRONCHOSCOPY: VIDEO:https://youtu.be/Iq74qwi5Aws
BAL : jt showshypereosinophillic cells , nucleoli prominent
on inguinal biopsy , it shows emperipolesis with histiocytosis confirmed as rosai- dolfman syndrome
DEFINITIVE DIAGNOSIS: rosai dorfman syndrome with splenomegaly.
TREATMENT
plan of treatment
.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
DEFINITIVE TREATMENT: Spleenectomy
MY QUESTIONS:
DOES PORTAL VEIN DILATION IS DUE TO PERIPORTAL LYMPHADENOPATHY/ Ncph?
as rosai dolfman syndrome causes generalized lymphadenopathy which in turn causes splenomegaly ?
why sister has spleenomegaly , no genetic predilection?
HALO SIGN DIFFERENTIALS?
hemorrhagic nodules of infectious origin (mucormycosis, candidiasis, tuberculosis, viral pneumonia, and invasive aspergillosis--the last being the most common cause of the CT halo sign); hemorrhagic nodules of noninfectious origin (Wegener granulomatosis, Kaposi sarcoma, and hemorrhagic metastases); tumor cell infiltration (bronchioloalveolar carcinoma, lymphoma, and metastasis with intra-alveolar tumor growth); and nonhemorrhagic lesions (sarcoidosis and organizing pneumonia)
EMPERIPOLESIS( a megakarocyte containing neutrophil) also SEEN IN
myelodysplastic syndrome, myeloproliferative neoplasm,chronic lymphocytic leukemia
CREDITS and reference:
https://raveen7795.blogspot.com/2022/11/14m-massive-splenomegaly.html
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