Haematologica 2003 - Supplements

103
Vascular amyloidosis induces severe gastrointestinal
bleeding in patients with monoclonal gammopathy
Laurent Garderet1, Nicolas Carbonell*2, Sylvie Lesage*1,
Francoise Isnard*1, Raoul Poupon*2, Albert Najman1
Department of Hematology1 and Gastroenterology2, Saint Antoine
Hospital, Paris, France.
Aim: Severe gastrointestinal bleeding is a rare event in patients
with monoclonal gammopathy (MG). Case reports have described
bleeding induced by hepatic failure, metastatic calcifications of
blood vessels, and gastric plasmocytoma. 15% of myeloma and
5% of MGUS have AL amyloidosis. Gastrointestinal involvement
in systemic AL amyloidosis is a common event but symptomatic
cases are rare. We report 5 patients with MG, from a single
institution during the last 3 years, presenting amyloidosis-induced
gastrointestinal hemorrhage. Patient characteristics: In each case,
a definite diagnosis was made by tissue biopsy and there was no
coagulopathy. Patient’s age ranges from 59 to 83 year old and
there were 3 women and 2 men. Three had multiple myeloma:
one stage I with 70% plasmocytes and IgGK=25g/l, one stage I
with 22% plasmocytes and IgGK=14g/l and one stage III with
19% plasmocytes and IgGL=41g/l. Two had MGUS: one with
IgGL=6.6g/l and IgAL=4g/l and one with IgGL=20g/l. Results:
The acute bleeding revealed the MG in two cases and occurred
during evolution in three. Hematemesis was the major symptom
in four patients and two needed intensive care management. One
patient had melena and rectorragy with acute colitis. For two
patients, endoscopic findings were: severe hemorrhagic gastritis
with white ulcerated nodules and prepyloric ulcerations in one
case, severe diffuse hemorrhagic ulcerations of the bulbe and
atrophic gastritis with microulcerations and a mucosal friability
which bleeded after biopsy in the other case. One patient had both
prepyloric ulcerations and diffuse ulcerative colitis. For another,
necropsy showed a massive gastric ulceration centred by a
punctured artery. A diagnosis of amyloidosis was done twice by
gastric, once rectal, once colic biopsy and once after necropsy.
All patients had amyloid deposition in the vessel walls with one
involving the whole body middle size arteries. Muscularis
mucosae was twice involved. Four of them died: two immediately
after massive hematemesis, one of kidney amyloidosis and
another of sudden cardiac arrest. In one multiple myeloma
patient, bleeding never resumed after chemotherapy with a
double autologous transplantation. Following treatment, that
patient had a normal colonoscopy but with persistent amyloidosis
in the colon vessel walls. Our patients are most likely the first set
of cases of gastrointestinal bleeding induced by amyloidosis in
MG. Gastric ulcerations were common endoscopic findings.
Amyloid hemorrhage is most often due to amyloid infiltration of
blood vessels and it carries a serious prognosis. In conclusion,
gastrointestinal bleeding in a patient with MG should induce a
diagnostic procedure for amyloidosis. More generally,
unexplained intestinal bleeding may be amyloidosis induced and
the physician should look for a MG.
104
An exceptional association of diffuse anaplastic
myeloma and microangiopathic anemia: a case report.
F.Taccone, V. Robin, A. Kentos, R. Maréchal, Y. Bouko,W.
Feremans.
Erasme hospital Université Libre de Bruxelles
A 70-year old man was admitted to the Haematology Department
because of fatigue and acute pain in the right forearm for three
weeks. Past medical history revealed orchitis four years before
actual presentation. Four months before admission, he presented
progressive bilateral paralysis of both inferior limbs and was
admitted with suspicion of Guillan-Barré syndrome. Complete
work-up showed demyelinating neuropathy, IgG-kappa
monoclonal band on plasma protein electrophoresis and moderate
light-chain proteinuria. Bone marrow biopsy revealed 10% of
plasmatocytes, and the caryotype was normal. Diagnosis of
peripheral polyneuropathy associated with monoclonal
gammopathy of undetermined significance (MGUS) was made
and the patient was successfully treated with high-dose
methylprednisolone and discharged to start a revalidation
program. He had progressive amelioration and began walking
without support. Blood values were normal at that time. At home,
treatment consisted of paroxetin, zolpidem and cholecalciferol.
At admission he complained of progressive asthenia with mild
exertional dyspnea. He could not sleep because of right arm pain
which developed next to the wrist two weeks before. He had tried
nonsteroidal anti-inflammatory drugs with no improvement of
symptoms. He noticed progressive weakness of both legs.
Vital signs were within the normal limits. Oxygen saturation was
94% while the patient was breathing room air.
Physical examination revealed left basal pulmonary
hypoventilation associated with dullness, peripheral bilateral leg
swelling with diffuse purpura, bilateral inferior limbs weakness
(4/5) with ankle and patellar hyporeflexy (1/4).
Blood tests showed anaemia, thrombocytopenia and renal failure.
There was proteinuria (1g/24h) consisting exclusively of kappalight
chain. Chest radiography showed left pleural effusion. Bone
marrow biopsy revealed massive infiltration by plasmatocytes
(58%); 5% of them stained positive for a kappa-light chain.
Caryotype analysis showed partial deletion of chromosome 13.
CT-scan of the arm was normal. Complete bone radiographic
study showed no lytic lesion. Diagnosis of multiple myeloma was
made and VAD-type chemotherapy was started. Because of
polypnea and light hypoxia, diagnostic thoracocentesis was made
and numerous monoclonal plasmocytic cells were found in the
pleural fluid.
On the second day of chemotherapy the patient complained of
diplopia with left sixth cranial nerve palsy. Cerebral CT-scan and
MRI showed no specific periventricular lesions. Nevertheless,
lumbar puncture showed plasmocytic cells and intrathecal
methotrexate was administered.
During chemotherapy, the patient developed Enterococcus
Faecalis septicaemia treated by intravenous ampicilline and
gentamycine. On the sixth day, haemolytic anaemia with
aggravation of renal failure and coma were noticed.
Blood tests showed the following: haemoglobin 7.3 g/dL (13-17
g/dL), platelets 20000/mm3 (150000-400000/mm3), LDH 4637
UI/dL (NR: 60-310 UI/dL), schistocytes > 8/1000 , creatinin 1.9
mg/dL (0.5-1.0 mg/dL), haptoglobin