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Karen Hansen, MD, FAAP
Primary Children's Medical Center
University of Utah
Salt Lake City, UT 84113
To the Editor.—
Rooms et al1 discuss central nervous system (CNS) hemophagocytic lymphohistiocytosis (HLH) as a confounder for abusive head trauma. Case 2 was our patient and was reviewed by one of the authors at the parents' request.
The 4-month-old child presented with seizures, obtundation, chest bruise, bilateral retinal hemorrhage (preretinal, intraretinal, and subretinal) extending from ora serrata to ora serrata, and vitreous hemorrhage. Computed tomography of the head showed acute parenchymal and subdural hemorrhage (parafalcine, tentorial, right frontal), and cerebral edema. Prothrombin time and liver enzymes were elevated, and anemia was present. Elevated triglycerides and conjugated bilirubin were noted but not initially recognized as suggestive of HLH. The parents denied trauma and significant disease, and abusive head trauma was suspected. A craniotomy specimen of the right frontal leptomeninges and subdural hemorrhage subsequently contained atypical hemophagocytic histiocytes, suggestive of HLH.
Autopsy revealed focal sparse histiocytic infiltrates of the leptomeninges and perivascular spaces without involvement of the parenchyma, giant cell hepatitis, and optic nerve subdural and subarachnoid hemorrhage. HLH infiltrates and necrosis were not present in the retinas or the optic nerve hemorrhages.
The literature describing the clinical presentation, natural history, and neuropathology of HLH is voluminous and was reviewed before certification of the cause of death.
We found no reports of HLH presenting with acute fatal intracranial hemorrhage. CNS hemorrhage is rare and is reported to be clinically insignificant. Focal retinal hemorrhages are described in only 2 patients with advanced disease.2,3 They did not resemble the retinal hemorrhages in our patient, which were of the type thought to be most specific for abusive head trauma.4 Several patients with extensive ocular HLH had no retinal hemorrhage.5–8
The patient's fatal brain edema, intracranial hemorrhage, and retinal hemorrhage are highly consistent with abusive head trauma and have not been described in HLH. Our opinion is that the patient died from abusive head trauma and that HLH was a comorbid condition. The same may also be true of case 1 in the Rooms et al article.1
Rooms et al1 illustrated CD-68-positive erythrophagocytic histiocytes in the subdural and ocular hemorrhages. All cells containing lysosomes express CD-68, are ubiquitous, and actively participate in healing. Demonstration of CD-68-positive cells is not proof that spontaneous hemorrhages occurred as the result of HLH.
The authors cited Miner et al9 as evidence that coagulopathy is not caused by CNS injuries due to abusive head trauma. Elevated prothrombin time/partial thromboplastin time and anemia have both been described in shaken infants.10
Case 3 had humeral periosteal new bone and tibial cortical irregularity. Osseous abnormalities are not reported in the HLH literature. These unexplained findings in a nonambulatory infant are suspicious for inflicted injury. A forensic investigation would have determined whether the abnormalities were HLH.
We agree with the authors that clinicians need to be diligent and informed when considering child abuse as a diagnosis. They must be equally diligent and informed in distinguishing between natural disease and inflicted injury. Children with chronic diseases are at increased risk for abuse,11 and infants with CNS manifestations of HLH may be fussy and difficult to care for.12 Careful consideration of the HLH disease process indicates that this disease will rarely if ever be a confounder for abusive head trauma.
REFERENCES
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