Sudden Fever

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					Case discussion                                                         J Indian Rheumatol Assoc 2004 : 12 : 31 - 34

Vikas Agarwal, Atul Sachdev, Ram Singh, Sabita Basu*
Departments of Medicine, Hematology & Transfusion Medicine, Government Medical College, Chandigarh

         A 52 years old man presented with sudden,           Roentgenogram of the chest and the skull were nor-
painless loss of vision in right eye for 1 day. He had       mal. Serum and urine electrophoresis did not reveal
moderate grade intermittent fever, night sweats and pain     monoclonal band. Computerized tomographic scan of
in both the knee joints and significant loss of weight       the head was normal. Visual evoked potential showed
(>8 kg) and appetite for 3 months and severe occipital       reversal of Chan pattern. No wave formation was no-
headache for 15 days prior to admission. There was           ticed on the right side. Ultrasound of the eye revealed
no history of stiffness of girdle muscles, temporal head-    widening of the nerves on both sides (right side 3.5
ache, amaurosis fugax and jaw claudication. Examina-         mm, left side 3.5-5.2 mm) with increased vasculature
tion showed pallor, and cervical lymphadenopathy. Ex-        near optic nerve head suggestive of optic neuritis. Ul-
amination of the right eye revealed absent perception        trasound abdomen showed enlarged liver and spleen
of light, acute flare with cells in the anterior chamber,    with para-aortic (peri-pancreatic) lymphadenopathy.
pigmented keratic precipitates on back of cornea, mul-       His vision improved completely, over 2 weeks, follow-
tiple retinal hemorrhages and exudates and anterior is-      ing therapy with prednisolone and a diagnostic proce-
chemic optic neuropathy. Left eye was normal. All the        dure was performed.
peripheral and central pulses were normally palpable.
There was no bruit, tenderness, loss of pulsation, over      Discussion:
any of the temporal or carotid arteries. Rest of the ex-             With the symptomatology of new onset occipi-
amination was unremarkable. Investigations revealed:         tal headache, high ESR, ischemic optic neuritis and pres-
hemoglobin 7.0 g/dl, total leukocyte count 3400/ l,          ence of constitutional symptoms in a male (>50 years),
erythrocyte sedimentation rate 65 mm, platelet count         temporal arteritis (TA) was a strong possibility. He ful-
76,000/ l and polymorphs 36%, lymphocytes 64% and            filled 3 of 5 ACR 1990 criteria for the diagnosis of
mild anisocytosis, hypochromia, polychromasia and            TA1.
normoblastemia 15/100 WBC and rouleaux formation                     The onset of TA tends to be gradual, but can
on peripheral blood film. Renal , liver function tests and   be abrupt and systemic symptoms are reported in 50%
urinalysis were normal. Rheumatoid factor was nega-          of the patients2. Fever may vary from low to moderate
tive and C-reactive protein was 107 mg/dl (normal            grade and may be the presenting manifestation. Head-
<10). Serology for HBsAg, anti HCV, antinuclear an-          ache is the most frequent symptom and occurs in two
tibody (ANA), anti-neutrophilic cytoplasmic antibody         thirds of patients3. It is frequently marked and tends to
ANCA), anti HIV-1 and -2 and VDRL was negative.              be located over the temporal or occipital areas. Per-
                                                             manent partial or complete loss of vision in one or both
Address for correspondence
                                                             eyes occurs in up to 20 percent of patients and is often
Vikas Agarwal
Senior Lecturer, Dept of Medicine                            an early manifestation of the disease2. Once established,
C1221, Sector 32B, Chandigarh, 160030                        visual impairment is usually permanent. Visual loss is
                                                   Vikas Agarwal et al

caused by ischemia of the optic nerve or tracts as a           phoma, CLL and HCL. Most of these disorders have
result of arteritis of the branches of the ophthalmic or       not been reported to cause optic neuritis except few
posterior ciliary arteries and, less commonly, by occlu-       case reports of HCL and CLL.
sion of the retinal arterioles2. The early funduscopic find-            Hairy cell leukemia (HCL) is known to present
ings consist of ischemic optic neuritis with a slight pal-     with pancytopenia and vasculitis11. It is an indolent B-
lor and edema of the optic disk and scattered cotton-          cell malignancy characterized by pancytopenia and sple-
wool patches and small hemorrhages as was seen in              nomegaly. It comprises of 2% of all the leukemias. It
our case.                                                      usually presents in patients over the age of 45 years
         A markedly elevated erythrocyte sedimentation         and present with constitutional symptoms, abdominal
rate is also a hallmark of giant-cell arteritis3. As part of   lump, recurrent infections, and anemia. Splenomegaly
the acute phase response in vasculitis leukocytosis and        is the most conspicuous examination finding, seen in
thrombocytosis was expected however our patient pre-           80% of the patients. Lymphadenopathy is rare and
sented with pancytopenia. Cytopenias without drug              serves to differentiate HCL from other LPD. Associa-
toxicity have not been reported in TA4.                        tion between HCL and polyarteritis nodosa like vas-
         Presence of pancytopenia and abdominal lym-           culitis was first noted by Hughes et al in 197912. Elkon
phadenopathy in our case was suggestive of underlying          et al13 had discussed 4 patients of HCL with vasculitis,
lymphoproliferative disease (LPD) or hematological             one of the patients was suspected to have temporal
malignancy. Given the short history, a possibility of acute    arteritis and two were reported to have temporal ar-
leukemia infiltrating into the optic nerve could not be        tery aneurysms. The patient with suspected diagnosis
ruled out however, acute leukemia (myeloblastic or lym-        of TA was a 53 year old male, diagnosed to have HCL
phoblastic) usually presents as optic disk edema5, reti-       1 year back and had undergone splenectomy 9 months
nal hemorrhages, retrolaminar leukemic infiltration6,          back. He presented nodular panniculitis on legs, pul-
enlargement of the optic nerves7, total retinal detach-        monary infiltrate and temporal artery aneurysms. The
ment8, or as isolated extramedullary relapse9. Our pa-         histology of the temporal artery revealed intimal thick-
tient had no leukemic cells in the peripheral blood and        ening, fragmentation of the internal elastic lamina and a
the fundus examination did not reveal features of leu-         mononuclear cell nitration. Giant cells were absent. He
kemic infiltration thus the possibility of acute leukemia      died 11 months after the development of the vasculitis.
seem remote; however it cannot be excluded defini-             Besides this there are no reports of TA like presenta-
tively.                                                        tion in HCL. There are very few reports of ocular in-
         Vasculitis as a complication of LPD is uncom-         volvement in HCL. Few cases with corneal deposits
mon and is usually of 2 types; cutaneous and systemic10.       and uveitis have been reported14. Only one case has
Cutaneous vasculitis is usually caused by lymphocytic          been reported with retinal vasculitis15. Robinson and
lymphoma, Cutaneous T-cell lymphoma, Hodgkin’s                 colleagues have reported a 73 year old man develop-
disease, angioimmunoblastic lymphadenopathy with               ing severe panuveitis in one eye and leukemic retin-
dysproteinemia, chronic lymphocytic lymphoma (CLL)             opathy in the other eye. He did not have hepato-sple-
and hairy cell leukemia (HCL). Systemic vasculitis is          nomegaly and lymphadenopathy but had anemia, rela-
usually caused by Hodgkin’s disease, lymphocytic lym-          tive neutropenia and high ESR (140 mm). In this pa-

                      A case of sudden onset loss of vision, fever, weight loss and pancytopenia

tient panuveitis did not respond to glucocorticoid
therapy. However, both the conditions improved mark-
edly following therapy with 2-chloro-deoxy-adenos-
ine16. Our patient had ischemic optic neuritis which re-
covered following glucocorticoid therapy, thus favor-
ing a possibility of vasculitis.
         Hasler and co-workers have reviewed the
prevalence and types of vasculitides associated with
HCL11. They have gathered information from 42 cases
reported till 1995. According to the clinical presenta-
tion and histopathologic findings the vasculitis in HCL
has been classified into 3 groups; PAN like vasculitis,       Figure: Peripheral blood smear showing hairy cells. (H & E
leukocytoclastic vasculitis and direct invasion of the ves-   staining, X 200)
sel wall by hairy cells. PAN like vasculitis has been
reported following splenectomy and was often preceded         Lesson:
by infection. On the other hand LCV appeared either                  In clinical presentation of vasculitis with
prior to or concomitantly with the diagnosis of HCL.          cytopenias, consider underlying HCL.
Considerable numbers of cases were associated with
Mycobacterium tuberculosis or atypical mycobacterial          References:
                                                              1.   Hunder GG, Bloch DA, Michel BA, et al. The American
infection. Hairy cell infiltration of vessel wall was noted
                                                                   College of Rheumatology 1990 criteria for the classification
in all the patients of HCL following splenectomy. It has           of giant cell arteritis. Arthritis Rheum 1990; 33:1122-8.
been hypothesized that cross reactivity between the           2.   Salvarani C, Cantini F, Boiardi L, Hunder G. Polymyalgia
                                                                   rheumatica and giant cell arteritis. N Engl J Med 2003;
hairy cells or antigens of infectious agents and endot-
                                                                   347: 261-71.
helium may contribute to vasculitis11.                        3.   Huston KA, Hunder GG, Lie JT, Kennedy RH, Elveback
         Procedure: Bone marrow aspiration and bi-                 LR. Temporal arteritis: a 25-year epidemiologic, clinical,
                                                                   and pathologic study. Ann Intern Med 1978; 88:162-7.
opsy revealed hypercellular marrow with sheets of
                                                              4.   Liozon F, Fourgnaud-Gaillard S, Bordessoule D, et al.
mononuclear cells with central nuclei and scanty to                Agranulocytosis caused by dapsone. Apropos of 3 cases.
moderate cytoplasm. Some of these cells show clefting              Review of the literature. Ann Med Interne (Paris). 1988;
of nuclear membrane. At places these mononuclear cells             139:469-75.
                                                              5.   Mayo GL, Carter JE, McKinnon SJ. Bilateral optic disk
have enclosed areas with red cell collections and posi-            edema and blindness as initial presentation of acute
tive bone marrow reticulin staining, suggestive of hairy           lymphocytic leukemia. Am J Ophthalmol. 2002; 134:141-2.
cell leukemia (Figure).                                       6.   Schocket LS, Massaro-Giordano M, Volpe NJ, Galetta SL.
                                                                   Bilateral optic nerve infiltration in central nervous sys-
         Presence of cervical and abdominal lymphad-               tem leukemia. Am J Ophthalmol. 2003;135:94-6
enopathy and absence of clinically palpable splenom-          7.   Madani A, Christophe C, Ferster A, Dan B. Peri-optic
egaly in our case was against the possibility of the HCL,          nerve infiltration during leukaemic relapse: MRI diagno-
                                                                   sis. Pediatr Radiol. 2000; 30:30-2.
initially. However, hepato-splenomegaly on USG ab-            8.   Primack JD, Smith ME, Tychsen L. Retinal detachment in
domen and characteristic hairy cells on bone marrow                a child as the first sign of leukemic relapse: histopathol-
examination confirmed HCL in our case.                             ogy, MRI findings, treatment, and tumor-free follow up. J
                                                       Vikas Agarwal et al

       Pediatr Ophthalmol Strabismus. 1995; 32:253-6.              14. Zak P, Chrobak L, Podzimek K, et al. An unusual course in
9.     Camera A, Piccirillo G, Cennamo G, et al. Optic nerve           hairy-cell leukemia with marked abdominal lymphaden-
       involvement in acute lymphoblastic leukemia. Leuk               opathy, leukemic infiltration of the cornea and skin
       Lymphoma. 1993; 11:153-5.                                       changes. Vnitr Lek. 1996; 42:463-6.
10.     Wooten MD, Jasin HE. Vasculitis and lymphoproliferative    15. Di Maria A, Redaelli C, Canevari A, Pagnucco G, Martinetti
       diseases. Semin Arthritis Rheum. 1996; 26:564-74.               M, Bianchi PE. Unilateral retinal vasculitis associated with
11.    Hasler P, Kistler H, Gerber H. Vasculitides in hairy cell       hairy cell leukaemia: immunogenetic study.
       leukemia. Semin Arthritis Rheum. 1995; 25:134-42.               Ophthalmologica. 1998; 212:355-7.
12.    Hughes GR, Elkon KB, Spiller R, Catovsky D, Jamieson I.     16. Robinson A, Eting E, Zeidman A, Djaldetti M, Mittelman
       Polyarteritis nodosa and hairy-cell leukaemia. Lancet.          M, Savir H. Ocular manifestation of hairy cell leukemia
       1979; 1:678.                                                    with dramatic response to 2-chloro-deoxy-adenosine. Am
13.    Elkon KB, Hughes GR, Catovsky D, et al. Hairy-cell leu-         J Ophthalmol 1996;121:97-9
       kaemia with polyarteritis nodosa. Lancet. 1979; 2:280-2.

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