FRCPath Haem Part 1 MCQ-Gen Haem 443

A 48-year-old man is admitted with a 4-week history of fever, drenching night sweats, weight loss and progressive confusion. He has received three courses of broad-spectrum antibiotics without clinical improvement.

Examination reveals:

• Temperature 39.5°C

• Splenomegaly (5 cm below costal margin)

Test	Result
Hb	78 g/L
WBC	1.8 × 10⁹/L
Neutrophils	0.7 × 10⁹/L
Platelets	42 × 10⁹/L
Ferritin	21,500 µg/L
Triglycerides	5.8 mmol/L
Fibrinogen	0.9 g/L
AST	245 U/L
ESR	7 mm/hr
EBV PCR	Positive

Bone marrow aspirate shows no evidence of haemophagocytosis.

An HScore is calculated at 210.

The ICU team is reluctant to commence immunosuppressive therapy because of concern regarding occult infection and the absence of haemophagocytosis on bone marrow examination.

Which ONE of the following is the most appropriate management decision?

A.

Exclude HLH because haemophagocytosis is absent and continue broad-spectrum antibiotics alone

B.

Await repeat bone marrow examination demonstrating haemophagocytosis before initiating therapy

C.

Perform soluble CD25 and NK-cell activity assays before making a diagnosis of HLH

D.

Initiate high-dose corticosteroids while continuing investigation and treatment of potential infectious triggers

E.

Initiate etoposide immediately because corticosteroids are ineffective in EBV-associated HLH