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n. hepatosplenomegalia, agrandamiento del hígado y del bazo.
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Hepatosplenomegaly was a consistent feature of this study, which is similar to that reported by R K Arora et al.
Descriptive parameters of the children (N=7) Parameter Case 1 Case 2 Case 3 Age and sex 11 years, F 14 years, F 7 mo, F Clinical features Fever, abdominal Fever, Fever pain abdominal pain, tachycardia, hypotension Hepatosplenomegaly Present Present Present Predisposing factors Malnutrition Malnutrition None Single/multiple Multiple Multiple Multiple abscesses Blood culture Burkholderia Sterile Sterile pseudomallei* Serology Negative Negative Negative (Widal, Weil-Felix, IgM scrub typhus) Abscess size 2 x 3 mm 21 x 15 mm 33 x 31 mm (maximum) Treatment IVA IVA IVA, USG- guided aspiration Leukocyte count, 19 500 15 000 19 000 cumm.
At the same time, her 45-day-old brother was diagnosed with FHL-2 (fever, hemocytopenia, hepatosplenomegaly, hypertriglyceridemia, and hyperferritinemia), with compound heterozygous mutations of c.1349C>T (p.
In addition, clinical suspicion for histoplasmosis can be delayed, because many symptoms of disseminated fungal infection and HLH overlap, including fever, hepatosplenomegaly, pancytopenia, and coagulopathy.
The combination of hepatosplenomegaly, fever, and pancytopenia should raise the suspicion for visceral leishmaniasis and result in prompt intervention.
The lymphadenopathy, hepatosplenomegaly, and bone lesions in sarcoidosis may also mimic metastatic disease.
Affected infants present at an early age, with vomiting, diarrhea, and massive hepatosplenomegaly. Feeding difficulties and malabsorption lead to malnutrition, growth retardation, and cachexia, which, together with the severe liver disease, contribute to the patient's early demise, generally in the first year of life [5, 6].
They share common clinical manifestations, including daily spiking fever, typical transient cutaneous rash, arthritis, lymphadenopathy, hepatosplenomegaly and serositis.
Storage disorder like Gaucher's disease can present like DCM but usually associated with hepatosplenomegaly.
H syndrome (OMIM # 602782), first described in 2008, is a rare autosomal recessive genodermatosis which is multisystemic and is primarily characterized by cutaneous hyperpigmentation, hypertrichosis, hepatosplenomegaly, hearing loss, heart anomalies, hypogonadism, short stature, hyperglycemia (insulin-dependent diabetes mellitus), and hallux valgus/flexion contractures.
[3] Massive hepatosplenomegaly as a presentation of ALL has not been reported previously in any patient population.