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Flashcards in HLH Deck (10)
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1
Q

Test characteristics of hemophagocytosis for HLH diagnosis

A

Not pathognomonic or specific for HLH. A review of 78 bone marrow aspirates that showed hemophagocytosis included 40 that were associated with diagnosis of HLH and 38 without an associated diagnosis of HLH;

2
Q

HLH clinical course

A

The natural history of untreated HLH syndrome is almost uniformly fatal.

3
Q

Lab features

A

Very high ferritin (median 2950) (high sensitivity + specificity) + anemia (92%) + thrombocytopenia (over 80%, median 69k) + elevated liver enzymes (3x) and bilirubin (nearly all) + Hypertriglyceridemia or hypofibrinogenemia (90%) + LDH/GGT elevated + coag abnormalities + renal dysfunction + hemophagocytosis on BMB aspirate (25-100%)

4
Q

Signs/symptoms

A

Persistent fever (95%) + splenomegaly (89%) + dyspnea (unknown) + severe hypotension + possibly rash + highly variable neurologic abnormalities (30%) + pulmonary involvement (40%)

5
Q

Diagnosis

A
  • No single clinical or laboratory feature has sensitivity and specificity to unequivocally diagnose HLH in adults.
  • 1 scoring system is available
  • Basically treat if clinical suspicion is high enough because mortality is so high.
6
Q

Treatment in general

A

HLH-94 protocol

7
Q

What does HLH-94 protocol consist of?

A

IF triggered by infection or rheumatologic condition –> treat trigger (eliminate stimulus)
Etoposide, dexamethasone
IF CNS disease –> intrathecal MTX and hydrocortisone
IF no improvement –> continue therapy as bridge to HCT

8
Q

Triggers for HLH

A

Infection
Rheumatologic conditions
Lymphoid malignancies

9
Q

treatment of EBV if associated with HLH

A

rituximab

10
Q

What are you looking for in bone marrow biopsy of suspected HLH patient?

A

hemophagocytosis

Decks in Hematology, oncology Class (218):