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Peripheral cytopenias are common in systemic lupus erythematosus (SLE), but bone marrow
involvement is rarely reported. Myelofibrosis is a rare disorder characterized by
reticulin fibrosis of the bone marrow, which usually occurs in response to clonal
proliferation of hematopoietic stem cells in myeloproliferative disorders. However,
bone marrow fibrosis has also been described in association with auto-immune diseases,
We will report here a new case of bone marrow fibrosis associated with SLE. We also
reviewed the 27 cases published in the English language literature, and will discuss
the clinical presentation, outcome, treatment, and pathophysiology of bone marrow
fibrosis occurring in association with SLE.
Over one half of patients were diagnosed concomitantly with bone marrow fibrosis and
SLE. Epidemiological, clinical and biological features of lupus were unremarkable.
Except for the presence of reticulin fibrosis, the findings from the bone marrow biopsies
proved highly variable. Overall mortality was about 14% but corticosteroid-based therapy
lead to clinical improvement and reverted bone marrow fibrosis in most cases. Data
on the usefulness of other immunomodulatory therapies are inconclusive.
SLE may be complicated by bone marrow involvement, of a likely autoimmune origin.
Bone marrow fibrosis occurring with SLE is probably similar to “primary autoimmune
myelofibrosis” and may respond to steroid and immunomodulatory therapies. Further
studies with standardised proofreading of bone marrow aspirations and biopsies are
needed to delineate the clinical and biological features of this rare complication
Primary myelofibrosis; Bone marrow; Systemic lupus erythematosus
Hematological abnormalities such as anemia, auto-immune hemolysis, leukopenia, lymphopenia
and thrombocytopenia are very common in systemic lupus erythematosus (SLE) (Beyan
2007). Blood cytopenias are among the criteria for SLE in the revised American College
of Rheumatology (ACR) (Hochberg
1997) guidelines and may occur as the first symptom of the disease. Most of these manifestations
are caused by increased peripheral destruction of blood cells associated with circulating
auto-antibodies. Occasional case reports and small series have documented bone marrow
abnormalities in patients with SLE, such as myelofibrosis, aplastic anemia, pure red
cell aplasia, and features suggestive of myelodysplastic syndromes, suggesting that
the bone marrow may also be a target organ in the disease. Bone marrow fibrosis is
defined by the deposition of reticulin fibres in the bone marrow stroma. Fibrosis
usually occurs in response to the clonal proliferation of hematopoietic stem cells
(Kuter et al.
2007). Sporadic reports have suggested that bone marrow fibrosis may be part of the SLE
disease spectrum, and the relationship between disease control and pancytopenia improvement
provides indirect evidence for a causal relationship between SLE and bone marrow fibrosis.
Auto-immune myelofibrosis may respond to immunosuppressive treatment with regression
of the fibrosis and regeneration of the normal marrow tissue (Pullarkat et al.
2003). However, bone marrow involvement in SLE has not been thoroughly studied and the
etiological mechanisms of this rare complication remain unclear. We report here a
case of bone marrow fibrosis associated with SLE. In order to improve the knowledge
on this rare but serious complication of SLE, we have also reviewed all previously
A 17 year-old woman with a history of polyarthralgias and photosentivity was referred
to the hospital in 1999 for the evaluation of a Raynaud’s phenomenon. Clinical examination
revealed no anomaly. She presented with neutropenia (leukocyte count 2.4 × 109/l with 1.1 × 109/l neutrophil granulocytes and 0.74 × 109/l lymphocytes) without anemia or thrombocytopenia. Antinuclear antibodies were present
(>1/1280 with a speckled fluorescence pattern). Anti-DNA antibodies were absent. Anti-U1-RNP
and anti-SSA antibodies were positive. Three years later (2002), she was admitted
to the hospital because of atypical eating disorder and psychotic behaviour. No evidence
for neuropsychiatric lupus was found despite an extensive work-up. The patient was
diagnosed with schizophrenia and treated with olanzapine. Nine years later (2011),
she was referred to the hospital for fatigue, fever and pancytopenia. With the exception
of extreme paleness, clinical examination was unremarkable. Her weight is normal.
Laboratory findings on admission showed the following values: leukocyte count 0,48 × 109/l with 0.28 × 109/l neutrophil granulocytes and 0.08 × 109/l lymphocytes, hemoglobin 2.9 g/dl, platelet count 15 × 109/l and reticulocyte count 3 × 109/l, low-normal level of folate, normal levels of vitamin B12 and ferritin. Anti-SSA,
anti-RNP 70 and anti-U1-RNP antibodies were positive. The anti-DNA antibody titer
was 76 UI/ml in ELISA [normal range < 10]. Direct Coombs’ test was negative. C3, C4,
and CH50 were respectively 1.18 g/l [normal range: 0.79–1.52], 0.12 g/l [normal range:
0.2–0.51] and 120%. Abdominal ultrasonography showed limit homogen splenomegaly (around
12 cm). Tear drop cells or leukoerythroblastic blood smear were not noted. Two attempts
to aspirate bone marrow at different sites were unsuccessful and yielded only dry
taps. Bone marrow biopsy showed hypercellular marrow (cellularity: 80%) with focal
lymphocytic infiltration, dysmyelopoiesis, erythrophagocytosis and grade 1–2 fibrosis.
JAK-2, MPL W515L/K, and calreticulin mutations were not screen. A diagnosis of SLE
with bone marrow involvement was retained. High dose methylprednisolone (500 mg/d)
was given for 3 days. Then hydroxychloroquine and prednisone (1 mg/kg) were started,
in addition to supportive care with blood transfusion and antibiotics. However, the
patient remained pancytopenic. Intravenous immune globulins (30 g/d) were given for
4 days with remarkable improvement. Hemoglobin level after 2 months was 11.8 g/dl,
platelet count was 175 × 109/l and leucocyte count was 3.27 × 109/l with 2.32 × 109/l neutrophil granulocytes and 0.49 × 109/l lymphocytes. Prednisone was continued for 17 months and gradually tapered. The
patient did not experience change in her mental status during the course of steroid
treatment. She remains in good physical health and still takes hydroxychloroquine
Review of published cases
An electronic search of the literature was performed via MEDLINE by crossing the key
words “systemic lupus erythematosus” AND [“bone marrow fibrosis” OR “myelofibrosis”].
We then examined additional references from the retrieved articles. The study period
ran from January 1975 through December 2013. Only papers written in English were reviewed.
Of the 27 cases retrieved from the English language literature and our case, 3 were
males and 25 females, giving a ratio of 1:9. The age range was 12 to 70 years with
a mean of 36 years and a median of 29 years. Fifteen patients received a concomitant
diagnosis of SLE and bone marrow fibrosis, and 13 patients who had been previously
diagnosed with SLE subsequently developed bone marrow fibrosis. In these 13 cases,
the onset of bone marrow fibrosis varied from 8 months to 13 years after SLE diagnosis,
with a mean of 5 years. Seven of these patients were under corticosteroids at the
time of the hematological complication, two patients were under hydroxychloroquine,
one had discontinued azathioprine at least 4 months before (Kiss et al.
2000) and one had started azathioprine two weeks before, but 4 weeks after discontinuing
the drug no improvement had been observed (Vora et al.
1998). In patients with previously diagnosed SLE, symptoms attributed to the disease before
bone marrow fibrosis occurred were rheumatologic symptoms (11/13), muco-cutaneous
symptoms (8/13), renal involvement (3/13), serositis (3/13), and seizures (1/13).
For all 28 patients, at the time of admission for cytopenias subsequently attributed
to bone marrow fibrosis, the physical findings suggestive of SLE were rheumatologic
symptoms (8/28), renal involvement (7/28), serositis (6/28) and muco-cutaneous symptoms
(4/28). Splenomegaly was found in 11/26 patients (2 patients had undergone splenectomy
due to immune thrombocytopenia), hepatomegaly in 11 patients, and lymph node enlargement
or small diffuse lymph nodes in 6 patients. The other major symptoms reported were
fever (17/28) and bleeding (15/28). Previous hematological history was often unclear,
but at least 2 patients had been diagnosed with immune thrombocytopenia before the
diagnosis of bone marrow fibrosis.
Thirteen of the 28 patients had pancytopenia (anemia: Hb <10 g/dl, leukopenia: WBC < 4 × 109/l, and thrombocytopenia: platelets < 150 × 109/l), 13/28 had bicytopenia (anemia or/and leukopenia or/and thrombocytopenia), 1/28
had thrombocytopenia and neutropenia without leukopenia and 1/28, only thrombocytopenia.
Neutropenia (<1,5 × 109/l) was observed in 10/12 (16 missing data) patients, lymphopenia in 4/9 (19 missing
data), hemolysis with hyper-reticulocytosis in one case and a positive direct Coombs’
test in 10/18 (10 missing data). Hemoglobin levels ranged from 13.1 to 2.7 g/dl (mean
7.3 g/dl), platelets from 341 to 1 × 109/l (mean 50 × 109/l), and leucocytes from 6.8 to 0.35 × 109/l (mean 3.4 × 109/l). Of the 24 patients with thrombocytopenia, 11 had deep (<20 × 109/l), 8 severe (<50 × 109/l), and 5 moderate thrombocytopenia. Tear drop cells or leukoerythroblastic blood
smear, two common finding in primary myelofibrosis, were noted in 14 patients.
For 22/28 patients, a “dry tap” occurred during bone marrow aspiration. All bone marrow
biopsies showed bone marrow fibrosis with variable increases in the amount of reticulin
fibers and fibroblasts. Grades of bone marrow fibrosis were not always specified.
Global marrow cellularity was variable, ranging from increased (12/28) to normal (11/28)
or decreased (5/28) without any increase in blasts. All elements including megakaryocytes
appeared morphologically normal. Megakaryocytes were increased or/and with clustering
in 13/28 patients and decreased in 1/28. Focal or massive lymphocytic infiltration
was observed in 4/28, plasmocytosis in 1/28 and erythroid hyperplasia in 2/28. Fifteen
patients underwent repeated bone marrow examination showing improvement, with reduction
in reticulin amounts in 12/15.
Antinuclear antibodies were found in all patients, anti-dsDNA in 12/28, anti-Ro/SSA
in 2/28, anti-histone in 1/28, and a low complement level in 18/28 patients. Antiphospholipid
antibodies were detected in 4/28 patients. Anti-platelet antibodies testing came out
positive in 3 patients and negative in 4.
Follow-up time ranged from a few months to years but was often unspecified. The overall
mortality was 14% (4/28). Two patients died within few days with no other treatment
than blood transfusions and antibiotics. Two other patients who died received only
prednisone as a specific treatment. Improvement was noted in 17/28 patients, transient
response with need for new treatment in 5/28, and no improvement in 2/28. Supportive
care with antibiotics and transfusions was explicitly mentioned for 8 patients. Two
patients received granulocyte colony stimulating factor (G-CSF).
Immunomodulatory therapies consisted in corticosteroids (26/28) (prednisone, prednisolone
or methylprednisolone), intravenous immune globulins (4/28) (Ramakrishna et al.
1995; Aharon et al.
1997; Sacre et al.
2009), plasma exchanges (2/28) (Borba et al.
1993; Vora et al.
1998), azathioprine (2/28) (Foley-Nolan et al.
1992; Kiss et al.
2000), cyclophosphamide (1/28) (Borba et al.
1993), cyclosporine (1/28) (Kiss et al.
2000), danazol (1/28) (Ramakrishna et al.
1995), colchicine (1/28) (Ramakrishna et al.
1995), vincristine (1/28) (Ramakrishna et al.
1995) and splenectomy (1/28) (Ramakrishna et al.
1995). Of 20 patients who received only corticosteroids, 16 improved and 4 did not. Four
patients who received cortisone concomitantly with azathioprine, intravenous immunoglobulin
or cyclosporine improved. One patient received cortisone and showed a transient response
but pancytopenia relapsed so she received danazol, vincristine, colchicine, intravenous
immunoglobulins, then underwent splenectomy and finally improved (Ramakrishna et al.
1995). Another patient was treated with plasma exchanges and cyclophosphamide following
a transient response to cortisone and improved (Borba et al.
1993). One patient received intravenous immune globulins after 3 weeks of corticosteroid
treatment without response, and a marked improvement occurred within the following
week (Aharon et al.
Primary myelofibrosis is considered as a clonal myeloproliferative disorder (Tefferi
2012). However some diseases such as infections, neoplasms and autoimmune diseases may
also induce bone marrow fibrosis. The term “myelofibrosis” is used in some contexts
to describe any increase in bone marrow stromal fibres, regardless of the associated
disease, and in other contexts to define a specific myeloid disorder (primary myelofibrosis)
(Kuter et al.
2007). The word “myelofibrosis” is therefore ambiguous, and in this article we have chosen
rather to use the term “bone marrow fibrosis”. Some authors suggest the importance
of distinguishing between increases in bone marrow reticulin and collagen. Above-normal
reticulin amounts are generally regarded as a nonspecific sign of bone marrow abnormality,
but may or may not be a sign of serious neoplastic disease. In contrast, increased
collagen is less common and is mainly seen in tumours metastatic to the bone marrow
or in the late stages of myeloproliferative diseases. Unlike increased reticulin,
it is not always reversible (Kuter et al.
2007). In most cases reported here, it was unclear if trichrome collagen stain and/or
reticulin stain were performed, and the type and amount of fibrosis were not reported
according to established grading scales (Kuter et al.
2007). Another issue is whether finding bone marrow reticulin fibrosis per se should prompt a diagnosis of autoimmune myelofibrosis in a patient with SLE. For
example, mild degrees of reticulin fibrosis can be observed in conditions such as
immune thrombocytopenia and may be found in many patients with lupus when routine
bone marrow biopsies are performed (Pereira et al.
1998). Moreover, some authors have reported cases of bone marrow fibrosis in patients
who do not have SLE or other well-defined autoimmune syndromes (Bass et al.
2001; Pullarkat et al.
2003). They have defined “primary autoimmune myelofibrosis” as a disorder characterized
by cytopenias with bone marrow lymphocyte infiltration and grade 3 - 4 reticulin fibrosis
of the bone marrow, lack of atypical bone marrow cells or osteosclerosis, absent or
mild splenomegaly, and the presence of auto-antibodies. In our review, the 28 retrieved
cases have been considered as fulfilling criteria for SLE, although lupus symptoms
and signs leading to the diagnosis of SLE were not always reported in detail by the
authors. Their clinico-pathological features were very similar to those of the reported
cases of “primary autoimmune myelofibrosis”. Thus we tend to believe that “autoimmune
myelofibrosis”, just like autoimmune cytopenias, may occur as an isolated disorder,
or as a feature of other autoimmune diseases including SLE. Finally, cases of aplastic
anemia have also been reported in SLE patients. We found 25 published cases in the
English language literature (Aplastic anemia as a feature of systemic lupus erythematosus.
In preparation). In these cases, the bone marrow biopsy showed marked hypocellularity,
but the absence of reticulin fibrosis was often not specified, and thus the differentiation
between “lupus bone marrow fibrosis” and “lupus aplastic anemia” is not always clear,
raising the question of the borderland between these two rare features of SLE (Cavalcant
The pathogenesis of bone marrow fibrosis remains incompletely understood, but appears
to be a relatively nonspecific response of fibroblasts to underlying cellular abnormalities.
Increased reticulin is the result of fibroblast proliferation, and increased collagen
synthesis or altered collagen turnover appear to be due to decreased collagenase release
from macrophages and neutrophils (Kuter et al.
2007). Several growth factors appear to be implicated. The platelet-derived growth factor
(PDGF), found in megakaryocytes and platelets, stimulates fibroblast growth (Kuter
2007). The transforming growth factor β (TGFβ) and epidermal growth factor (EGF) are known
to promote collagen synthesis (Le Bousse-Kerdilès et al.
2008). Immunological abnormalities may be involved in the pathogenesis. The increased
circulating immune complexes and auto-antibodies that are present in SLE may act on
megakaryocyte Fc receptors and release growth factors to promote marrow fibrosis.
Some authors have suggested that both auto-antibodies against CD34+ stem cells and
cytotoxic T cells may initiate and perpetuate damage to the bone marrow (Kiss et al.
2000). An increase in leucocyte apoptosis and impaired clearance of apoptotic cells has
also been observed in patients with SLE. These apoptotic bodies were observed in the
bone marrow of patients with SLE, while they are not typically seen in normal bone
marrow. Delayed apoptotic cell clearance leads to prolonged exposure of auto-antigens
and predisposes to antibody production (Hepburn et al.
2007). Furthermore, in the bone marrow of patients with bone marrow fibrosis and SLE,
megakaryocyte counts are often above normal or normal. Therefore thrombocytopenia
may result at least partly from an increased destruction of the platelets rather than
a decreased production caused by bone marrow fibrosis. An association between immune
thrombocytopenia (to which bone marrow dysfunction is increasingly believed to contribute
2009)) and bone marrow fibrosis has been observed in 3 of the 28 reported cases.
The JAK2 V617F mutation, associated with primary myeloproliferative disorders, is
present in up to one half of the patients with primary myelofibrosis (Tefferi et al.
2012). Some authors suggest a thorough search for auto-immunity in the absence of the
mutation (Sacre et al.
Clinical and biological presentation
Primary myelofibrosis is diagnosed relatively late in life (median age is 66 years)
and is more common in males (ratio 3:2) (Tefferi et al.
2012). Bone marrow fibrosis occurring with SLE is diagnosed earlier (median age is 29 years)
and is very uncommon in males (ratio 1:9). In 15/28 cases, the diagnosis of SLE and
bone marrow fibrosis were made simultaneously. However, in 5 of these cases a number
of symptoms and signs (such as arthralgias, alopecia, proteinuria) were suggestive
of undiagnosed yet pre-existing SLE (Daly and Scott
1983; Matsouka et al.
1989; Inoue et al.
1992; Paquette et al.
1994; Pillai et al.
2009). Some authors suggest that autoimmune disorders, including SLE, may be considered
in cases of bone marrow fibrosis in patients whose spleen is not enlarged (Pullarkat
2003; Sacre et al.
2009), but 10/26 patients in our review had splenomegaly. Moreover, in primary myelofibrosis,
the clinical finding of splenomegaly is associated with collagen, but not reticulin
fibrosis (Thiele and Kvasnicka
2006). Fifteen patients underwent repeated bone marrow examinations showing improvement,
with a reduction in reticulin in 12/15. This suggests that reticulin fibrosis (and
maybe even collagen fibrosis) can be reversed if the underlying disease is treated
(Pereira et al.
Outcome and treatment
Although it is likely that negative outcomes are less frequently reported in case
reports, for which follow up data may be lacking, and that consequently the overall
mortality may be higher than the 14% documented from this review, this mortality rate
suggests a more favourable course for SLE-associated bone marrow fibrosis than for
primary myelofibrosis (Tefferi et al.
2012). Interestingly, bone marrow fibrosis occurring with SLE appears to often respond
to corticosteroids, unlike primary myelofibrosis. Plasma exchanges seem to have no
efficacy. Intravenous immune globulins were used for 4 patients only (Ramakrishna
1995; Aharon et al.
1997; Sacre et al.
2009), and proved to be efficient in at least one patient (Aharon et al.
1997), as it was the case for the patient we managed. None of the 28 patients received
rituximab. None received an allogeneic hematopoietic stem cell transplant.
SLE may be complicated by bone marrow fibrosis, which is likely to be of autoimmune
origin. This feature may be more common than previously thought, with cases being
incorrectly characterized as blood peripheral cytopenias in patients previously diagnosed
with SLE, and cases being misdiagnosed with primary myelofibrosis in patients not
previously diagnosed with SLE. We think that in patients with SLE, cytopenias should
be confirmed by bone marrow aspiration, and by bone marrow biopsy in atypical or refractory
cases. Moreover, autoimmune myelofibrosis or SLE-associated bone marrow fibrosis should
be considered in cases of primary myelofibrosis with atypical features such as young
age and female sex, absence of spleen enlargement, or absence of JAK2 V617F mutation,
because this condition seems amenable to efficient treatment. High-dose corticosteroid
therapy with or without intravenous immune globulins should be the first-line therapy.
In order to improve knowledge of bone marrow involvement in SLE, we have established
a French registry, with centralized proofreading of bone marrow aspirations and biopsies.
We hope to achieve a sufficient sample size for epidemiological and clinical research
on this unusual feature of lupus.
Written informed consent was obtained from the patient for the publication of this
The authors declare no conflict of interests.
All authors participated and drafted the manuscript. All authors read and approved
the final manuscript.
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