Clinical Otolaryngology 1977, 2, 327-338
0tolaryngological disturbances in Waldenstrom’s macroglobulinaemia M E H E R W E L L S , L. M I C H A E L S * AND D. G . W E L L S S.E. Kent, Canterbury and Thanet Group of Hospitals, Kent, and Institute of Lar.yngoloLqy and Otology, London
Accepted f or publication 27 May 1977 WELLSM., MICHAELS L. & WELLSD.G. (1977) Clinical Otolaryngology 2, 327-338 Otolaryngological disturbances in Waldenstrom’s macroglobulinaemia Four cases of Waldenstrom’s macroglobulinaemia (WM) are described in which otolaryngeal symptoms were present. Symptoms in WM are related to (a) bleeding diathesis and (b) hyperviscosity of the blood. The bleeding diathesis was manifested as epistaxis in z cases. In one case which came to postmortem, histological examination of the temporal bone showed complete disruption of the labyrinth by haemorrhage. Hyperviscosity probably was the source of hearing loss which took place in all 4 patients and had the following characteristics: (a) sudden onset (b) cochlear type (c) bilateral (d) sequential involvement of the two sides (e) improvement on treatment of the disease with alkylating agents or plasmapheresis. Early diagnosis of WM is thus important. Keywords macroglobulinaemia epistaxis sudden hearing loss temporal bone pathology
Introduction
Waldenstrom’s macroglobulinaemia (WM) is a primary plasma cell dyscrasia in which there is produced an excess of monoclonal macroglobulin of the IgM variety. This results in an increased blood viscosity which gives rise to circulatory disturbances caused by an increased resistance of the blood to flow. Such disturbances are prominent in the fundi where haemorrhages and exudates are often present and bulges and constrictions are seen in the retinalveins. Hyperviscosity vascular changes are also frequent in the central nervous system, resulting in a variety of neurological manifestations. As a result of the formation of complexes between macroglobulins and specific clotting factors, such as Factor VIII, a bleeding diathesis is a feature of WM and this is further complicated by interference of the macroglobulins with platelet function. One of the neurological disturbances of the hyperviscosity syndrome may be sudden hearing loss and the bleeding diathesis may lead to epistaxis so that cases of WM may be referred to the otolaryngologist or may present to him before a diagnosis is made. In this paper we describe 4 cases of WM in which otolaryngeal symptoms were present. In Case 4 the patient died and the temporal bone was available for pathological examination.
’
* Request for reprints to: Professor L. Michaels, Institute of Laryngology and Otology, 330 Grays Inn Road, London WCIX 8EE. 327
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M E H E R W E L L S , L. M I C H A E L S A N D D . G . W E L L S
Case histories CASE I
A man (A.R.) aged 73, was admitted with a history of occasional epistaxis of 3 years’ duration, haematuria for 2 months and impairment of hearing of sudden onset for 3 weeks. Initially the impairment of hearing was left-sided and later affected the right ear. He also complained of bilateral tinnitus. There was no history of otalgia, otorrhoea or vertigo.
Figure I Fundal appearances in left eye of Case I . Note the engorgement and slight beading of retinal veins particularly above the optic disk. Minute haemorrhages arc present to the medial side (left on photograph) of the optic disc.
The only positive findings on medical examination were those of bilateral inguinal lymphadenopathy and engorgement of the retinal vessels with haemorrhage (Figure I). Two weeks after hospitalization he was referred to the E N T Clinic from the Haematology Clinic with a diagnosis of WM: the diagnosis having been made on the basis of characteristic bone marrow appearances and serum electrophoretic and immunophoretic studies (Table I). On examination there was a bleeding point in the left Little’s area, The pharynx, nasopharynx and larynx were normal. The tympanic membranes were intact and mobile. Rinne’s test was
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MEHER WELLS, L. MICHAELS AND D . G . WELLS
positive on both sides and Weber’s not lateralized. An audiogram revealed a bilateral sensorineural hearing loss of 80-90 dB on the right and 60-80 dB on the left (Figure 2 ) . Further tests, in the form of tone decay and recruitment and speech audiogram, showed the hearing loss to be cochlear in origin. Tympanometry was normal. He was treated for I week with Chlorambucil 10mg daily and Prednisone I 5 mg tds. while awaiting plasmapheresis. Subsequently he had Prednimustine 10mg daily for 5 days and 2 weeks after plasmapheresis he had Chlorambucil 10mg daily and Prednisone 15 mg tds. He was reviewed 6 weeks after commencement of initial treatment when there was an objective improvement in his hearing. At further follow-ups according to the patient his hearing had returned to its original level (Figure 2 ) . The concentration of the monoclonal IgM however actually increased following treatment.
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0tolaryngological disturbances in Waldenstrom’s macroglobulinaemia M E H E R W E L L S , L. M I C H A E L S * AND D. G . W E L L S S.E. Kent, Canterbury and Thanet Group of Hospitals, Kent, and Institute of Lar.yngoloLqy and Otology, London
Accepted f or publication 27 May 1977 WELLSM., MICHAELS L. & WELLSD.G. (1977) Clinical Otolaryngology 2, 327-338 Otolaryngological disturbances in Waldenstrom’s macroglobulinaemia Four cases of Waldenstrom’s macroglobulinaemia (WM) are described in which otolaryngeal symptoms were present. Symptoms in WM are related to (a) bleeding diathesis and (b) hyperviscosity of the blood. The bleeding diathesis was manifested as epistaxis in z cases. In one case which came to postmortem, histological examination of the temporal bone showed complete disruption of the labyrinth by haemorrhage. Hyperviscosity probably was the source of hearing loss which took place in all 4 patients and had the following characteristics: (a) sudden onset (b) cochlear type (c) bilateral (d) sequential involvement of the two sides (e) improvement on treatment of the disease with alkylating agents or plasmapheresis. Early diagnosis of WM is thus important. Keywords macroglobulinaemia epistaxis sudden hearing loss temporal bone pathology
Introduction
Waldenstrom’s macroglobulinaemia (WM) is a primary plasma cell dyscrasia in which there is produced an excess of monoclonal macroglobulin of the IgM variety. This results in an increased blood viscosity which gives rise to circulatory disturbances caused by an increased resistance of the blood to flow. Such disturbances are prominent in the fundi where haemorrhages and exudates are often present and bulges and constrictions are seen in the retinalveins. Hyperviscosity vascular changes are also frequent in the central nervous system, resulting in a variety of neurological manifestations. As a result of the formation of complexes between macroglobulins and specific clotting factors, such as Factor VIII, a bleeding diathesis is a feature of WM and this is further complicated by interference of the macroglobulins with platelet function. One of the neurological disturbances of the hyperviscosity syndrome may be sudden hearing loss and the bleeding diathesis may lead to epistaxis so that cases of WM may be referred to the otolaryngologist or may present to him before a diagnosis is made. In this paper we describe 4 cases of WM in which otolaryngeal symptoms were present. In Case 4 the patient died and the temporal bone was available for pathological examination.
’
* Request for reprints to: Professor L. Michaels, Institute of Laryngology and Otology, 330 Grays Inn Road, London WCIX 8EE. 327
328
M E H E R W E L L S , L. M I C H A E L S A N D D . G . W E L L S
Case histories CASE I
A man (A.R.) aged 73, was admitted with a history of occasional epistaxis of 3 years’ duration, haematuria for 2 months and impairment of hearing of sudden onset for 3 weeks. Initially the impairment of hearing was left-sided and later affected the right ear. He also complained of bilateral tinnitus. There was no history of otalgia, otorrhoea or vertigo.
Figure I Fundal appearances in left eye of Case I . Note the engorgement and slight beading of retinal veins particularly above the optic disk. Minute haemorrhages arc present to the medial side (left on photograph) of the optic disc.
The only positive findings on medical examination were those of bilateral inguinal lymphadenopathy and engorgement of the retinal vessels with haemorrhage (Figure I). Two weeks after hospitalization he was referred to the E N T Clinic from the Haematology Clinic with a diagnosis of WM: the diagnosis having been made on the basis of characteristic bone marrow appearances and serum electrophoretic and immunophoretic studies (Table I). On examination there was a bleeding point in the left Little’s area, The pharynx, nasopharynx and larynx were normal. The tympanic membranes were intact and mobile. Rinne’s test was
330
MEHER WELLS, L. MICHAELS AND D . G . WELLS
positive on both sides and Weber’s not lateralized. An audiogram revealed a bilateral sensorineural hearing loss of 80-90 dB on the right and 60-80 dB on the left (Figure 2 ) . Further tests, in the form of tone decay and recruitment and speech audiogram, showed the hearing loss to be cochlear in origin. Tympanometry was normal. He was treated for I week with Chlorambucil 10mg daily and Prednisone I 5 mg tds. while awaiting plasmapheresis. Subsequently he had Prednimustine 10mg daily for 5 days and 2 weeks after plasmapheresis he had Chlorambucil 10mg daily and Prednisone 15 mg tds. He was reviewed 6 weeks after commencement of initial treatment when there was an objective improvement in his hearing. At further follow-ups according to the patient his hearing had returned to its original level (Figure 2 ) . The concentration of the monoclonal IgM however actually increased following treatment.
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