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Intravitreal Hemoglobin Spherulosis: A Rare Complication of Subretinal Hemorrhage
Arch Ophthalmol. 2002;120:85-87.
Hemoglobin spherulosis, a rare histological finding, represents a spherical
degradation product of hemoglobin. It has been described in a single report
as a vitreous hemorrhage originating from the subretinal space. We describe
an immunosuppressed patient with chronic myelogenous leukemia (CML) and associated
thrombocytopenia, with bilateral white intraretinal and subretinal hemorrhages,
along with an overlying vitreous hemorrhage. Cytologic examination of a vitrectomy
specimen demonstrated hemoglobin spherulosis. This represents only the second
report of this phenomenon.
Three unusual sequelae of longstanding vitreous hemorrhage are cholesterolosis
bulbi (synchisis scintillans), the formation of tubular vitreous cylinders,
and hemoglobin spherulosis.1 The last entity
represents a spheroidal degradation product of erythrocytes and was first
described by Grossniklaus et al2 in an eye
with a vitreous hemorrhage secondary to an idiopathic choroidal neovascular
membrane. Spherulosis was so named because of its histologic resemblance to
myospherulosis, an inflammatory condition characterized by saclike clusters
of degenerating erythrocytes that have been physically altered by exposure
to a foreign substance — typically an antibiotic-containing ointment.3-5
The purpose of this article is to document the clinical and pathological
correlation of hemoglobin spherulosis in an eye with bilateral white intraretinal
and subretinal hemorrhages, along with an overlying vitreous hemorrhage of
a patient with CML.
Report of a Case
A 39-year-old African American man experienced decreased vision, with
a greater degradation of vision in the right eye than in the left eye throughout
one month. His medical history was significant for CML with associated anemia
(hemoglobin level, 8.1 g/dL) and thrombocytopenia (22 x 103/µL),
though the disease was believed to be in remission. On ocular examination,
his uncorrected visual acuity was counting fingers at 3 ft OD, and 20/40 OS.
External and anterior segment examinations were unremarkable. Intraocular
pressure was within normal limits, and there was neither iris rubeosis nor
anterior chamber inflammation.
Dilated ophthalmoscopic examination in the right eye revealed prominent
brown vitreous cells, which obscured the retinal details and prevented fundus
photography. With indirect ophthalmoscopy, the optic disc margins appeared
sharp and flat. Large white intraretinal and subretinal hemorrhagic infiltrates
were visible in the mid periphery of the retina. Examination of the left eye
was similar, though there was less prominent overlying vitreous hemorrhage
(Figure 1).
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Figure 1. Ophthalmoscopic appearance of
the left eye depicting white intraretinal and subretinal hemorrhages in the
temporal quadrant (A) and in the inferonasal quadrant (B).
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The differential diagnosis included bilateral leukemic infiltrates,
endogenous endophthalmitis, and/or possible retinal opportunistic infection.
The patient underwent diagnostic and therapeutic pars plana vitrectomy in
his right eye, with intravitreal injection of 1.0 mg of vancomycin hydrochloride,
2.25 mg of ceftazidime, and 5.0 µg of amphotericin B. Vitreous cultures
for bacteria and fungi were negative, and cytology was negative for leukemic
cells. Following vitrectomy, his visual acuity improved to 20/40 OD.
Both undiluted vitreous and the vitrectomy cassette were processed using
the thin-prep technique, and the slides were stained with the Papanicolau
and Diff-Quik (Dade-Behring, Deerfield, Ill) methods. Cytologic examination
showed fragmented erythrocytes, ranging in diameter from 2 to 4 µm.
Larger (10-12 µm) dark brown globules were observed, indicative of hemoglobin
spherulosis (Figure 2). Rare inflammatory
cells were identified. Neither tumor cells nor microorganisms were observed.
Because of the relatively small amount of material, no additional studies
(special stains and electron microscopy) could be performed.
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Figure 2. Vitrectomy specimen processed
by the thin-prep technique, displaying 2- to 4-µm fragmented erythrocytes
and intermixed with large dark brown globules, the largest ranging from 10
to 12 µm in diameter (arrow indicates hemoglobin spherulosis) (Papanicolaou
stain, original magnification x100).
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Comment
McClatchie et al6-7
in 1969 reported several East African patients with inflammatory nodules involving
muscular and subcutaneous tissues. Histologically, the lesions represented
granulomatous inflammation surrounding "bags" of spherules, each spherule
being slightly larger than an erythrocyte. This condition was given the descriptive
term myospherulosis.
Eight years later, Kyriakos3 used
the term to describe a histologically similar inflammatory condition of the
nose, paranasal sinuses, and middle ear. His work, as well as the work of
of subsequent investigators4-5
demonstrated the contents of the sac-like structures to be erythrocytes altered
by a foreign substance, typically an antibiotic-containing ointment. Today,
myospherulosis is often a surgical complication and has been described in
numerous tissues, including the upper eyelid8
and caruncle.9
In 1988, Grossniklaus et al2 described
a unique hemoglobin degradation product recovered from a vitreous hemorrhage
in an eye with an idiopathic choroidal neovascular membrane. They named the
condition hemoglobin spherulosis because of its histologic
resemblance to myospherulosis. The mechanism of the spherule formation is
unknown, although they hypothesized that the initial subretinal location of
the blood may have played a role.
Gass10-11 has described
a yellow-brown material in the vitreous following hemorrhagic detachment of
the retina and retinal pigment epithelium. He proposed that this material
was composed of hemoglobin rather than whole blood, and he noted that the
material may also stain the iris stroma, resulting in heterochromia in a lightly
pigmented patient. The mechanism of anterior migration of subretinal hemoglobin
may be similar to that which causes corneal blood staining following a hyphema.
Since his initial publication, Grossniklaus has noted 2 additional cases,
although these were not formally reported.2
Our patient, therefore, represents only the second reported case of intravitreal
hemoglobin spherulosis in the literature. It is significant that in our patient,
the vitreous hemorrhage also seems to have originated in the subretinal space,
which may explain the pathogenesis of the spherule formation. Our patient
had at least 2 risk factors for the vitreous hemorrhage, including the underlying
CML, along with thrombocytopenia. With the white intraretinal and subretinal
hemorrhages, it was presumed that the intraocular hemorrhages had been present
for an extended period.
As discussed previously, 3 uncommon complications of longstanding vitreous
hemorrhage are cholesterolosis bulbi (synchisis scintillans), the formation
of tubular vitreous cylinders, and hemoglobin spherulosis.2
This last entity, which may be related to degenerative changes occurring to
hemoglobin as it migrates from the subretinal space into the vitreous cavity,
seems to be a poorly recognized phenomenon. Further studies are needed to
support this hypothesis. The full ocular significance of this phenomenon is
not fully known, though it may lead to a much more prolonged spontaneous clearance
of hemorrhage from the vitreous cavity. We therefore recommend that vitrectomy
specimens obtained from eyes with breakthrough subretinal hemorrhages be cytologically
evaluated to rule out hemoglobin spherulosis.
Unfortunately, 6 months following the pars plana vitrectomy surgery,
the patient experienced a relapse of his CML. The patient died due to complications
of pneumonia. An autopsy was not performed.
AUTHOR INFORMATION
Supported in part by a grant from the Retina Research Foundation, Houston,
Tex, and by an unrestricted grant from Research to Prevent Blindness Inc,
New York, NY.
Dr Schwartz is currently with the Department of Ophthalmology at the
Medical College of Virginia Campus of Virginia Commonwealth University, Richmond.
Stephen G. Schwartz, MD;
William F. Mieler, MD;
Eric R. Holz, MD;
Bita Esmaeli, MD;
Paul S. Ragsdale, CRA;
Ramon L. Font, MD
Houston, Tex
Corresponding author: William F. Mieler, MD, Cullen Eye Institute,
Baylor College of Medicine, 6565 Fannin St, NC-205, Houston, TX 77030 (e-mail: wmieler{at}bcm.tmc.edu).
REFERENCES
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1. Spraul CW, Grossniklaus HE. Vitreous hemorrhage. Surv Ophthalmol. 1997;42:3-39.
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2. Grossniklaus HE, Frank KE, Farhi DC, Jacobs GJ, Green WR. Hemoglobin spherulosis in the vitreous cavity. Arch Ophthalmol. 1988;106:961-962.
ABSTRACT
3. Kyriakos M. Myospherulosis of the paranasal sinuses, nose and middle ear: a possible
iatrogenic disease. Am J Clin Pathol. 1977;67:118-130.
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7. McClatchie S, Bremner AD. Unusual subcutaneous swellings in African patients. East Afr Med J. 1969;46:625-633.
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sinus surgery. Am J Rhinol. 1997;11:345-347.
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9. Kini U, Babu MK. Ocular spherulocystosis. J Clin Pathol. 1996;49:857-858.
FREE FULL TEXT
10. Gass JDM. Pathogenesis of disciform detachment of the neuroepithelium, III: senile
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11. Gass JDM. Stereoscopic Atlas of Macular Diseases: Diagnosis
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SECTION EDITOR: W. RICHARD GREEN, MD
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