Update: PUO case
8/12/2009 09:25:00 PM | Author: fadzly
This post is a follow up on a case which i discuss earlier. In case if you wanna know a bit about the patient history or you are lost, care to click here.

Patient currently on week 2 and currently on day 4 of oral augmentin course change from previous IV augmentin due to development of rashes based on Prof MT Koh saying that the cause of the rashes which raised on the patient trunk and the limbs could be from the antibiotic given. I'm start to think, patient was already on IV augmentin for 12 days and if the rashes really due to the antibiotics given, shouldn't it comes early? And if the patient really have the rash problem due to antibiotic, is the route of administration of the drug makes the rash goes away eventhough it using the same antibiotics? but anyway, the rashes was still there for the past 3 days.

Currently, oncology team lead by Prof MT Koh, makes the working diagnosis as Haemophagocytic Lymphohistiocytosis, which is a rare disorder of the immune system. The disease clinically manifests as fever, hepatosplenomegaly, pancytopenia(which the patient's platelet is normal), lymphadenopathy, and rash(and no rashes initially) often comprise the initial presentation.

Hemophagocytic Lymphohistiocytosis

The diagnostic criteria set forth by the Histiocyte Society for inclusion in the International Registry for Hemophagocytic Lymphohistiocytosis (HLH) is as follows. All 5 criteria must be met to establish a diagnosis of hemophagocytic lymphohistiocytosis:

* Fever - Seven or more days of a temperature as high as 38.5°C (101.3°F)
* Splenomegaly - A palpable spleen greater than 3 cm below the costal margin
* Cytopenia - Counts below the specified range in at least 2 of the following cell lineages:
o Absolute neutrophils less than 1000/µL
o Platelets less than 100,000/µL
o Hemoglobin less than 9.0 g/dL
* Hypofibrinogenemia or hypertriglyceridemia - (1) Fibrinogen less than 1.5 g/L or levels greater than 3 standard deviations below the age adjusted reference range value or (2) fasting triglycerides greater than 2 mmol/L or levels greater than 3 standard deviations above the age-adjusted reference range value
* Hemophagocytosis - Must have tissue demonstration from lymph node, spleen, or bone marrow without evidence of malignancy
* Rash - Skin findings in more than half of patients;1 scaly and waxy lesions; rashes on the scalp and behind the ear
* Other - Swollen or hemorrhagic gums that can result in tooth loss; feeding problems (especially prominent in infants); abdominal pain, vomiting, diarrhea, and weight loss

Light microscopic image of bone marrow showing stromal macrophages containing numerous red blood cells in their cytoplasm


I don't know why they were searching for all those rare things. If i answer this HLH in my long case final exam, sure GG. They are currently planning to have some blood investigations to confirm the HLH by doing fasting TG, fibrinogen, and waiting for trephine report.

When i ask one of the doctor there, why would they haven't done their lymphnode biopsy, the answer somewhat like this
"This is a generalised lymphadenopathy. How could we biopsy all the lymphnodes?????"
I don't know wether its true or not, but currently there is no plan for lymphnode biopsy yet.

A big suprise where today was the first time the patient is 24 hour free from fever. Maybe an oral antibiotic is working after all..
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