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CASE REPORT
A rare form of anemia in systemic lupus erythematosus
C.A. Mansoor, R. Narayan
Department of Internal Medicine, M.E.S. Medical College, Perinthalmanna, Kerala, India
SUMMARY
Mechanisms responsible for anemia in systemic lupus erythematosus (SLE) can be immune or non-immune.
A 27-year-old previously healthy woman was admitted with echymotic patches over the lower limbs for six months, multiple joint pain and fatigue for 2 months. She had severe pallor and multiple echymotic patches over the lower limbs. She was diagnosed with SLE with pernicious anemia and iron deficiency anemia. The rare as- sociation of SLE with pernicious anemia was reported previously in few patients. Treatment of SLE along with B12 supplementation is necessary for such patients. Since etiology for anemia in SLE can be of various kinds, a detailed workup for identifying the underlying mechanism is necessary.
Key words: Lupus; Perinicious anemia.
Reumatismo, 2017; 69 (3): 119-121
n INTRODUCTION
Hematological abnormalities are com- mon in systemic lupus erythematosus (SLE). Anemia can be seen in about 50%
of patients, with anemia of chronic disease being the most common form (1). Various immune and non-immune mechanisms are responsible for anemia in SLE and include inflammation, renal insufficiency, blood loss, dietary insufficiency, medications, haemolysis, infection, hypersplenism, my- elofibrosis, myelodysplasia, and aplastic anemia (2, 3). Pernicious anemia and SLE
are both disorders of autoimmune etiology.
Coexistence of SLE with other autoim- mune disorders is common but association of pernicious anemia and SLE is rare.
n CASE REPORT
A 27-year-old previously healthy woman was admitted with echymotic patches over lower limbs for six months, multiple joint pain and fatigue for 2 months. She had menorrhagia for 5 years. She denied his- tory of weight loss, had no sick contacts and had no history of addictions. She had
Corresponding author C.A. Mansoor
Department of General Medicine, M.E.S. Medical College, Perinthalmanna, Kerala 679338, India
E-mail: drcamans@gmail.com
Figure 1 - A and B) Peripheral smear showing moderately hypochromic RBCs, marked aniso- poikilocytosis with microcytes, few hypersegmented neutrophils and severely reduced platelet count.
Reumatismo, 2016; 69 (3): 119-121
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being the most common form (1). Various
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being the most common form (1). Various immune and non-immune mechanisms are
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immune and non-immune mechanisms are responsible for anemia in SLE and include
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responsible for anemia in SLE and include inflammation, renal insufficiency, blood
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inflammation, renal insufficiency, blood loss, dietary insufficiency, medications,
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loss, dietary insufficiency, medications, haemolysis, infection, hypersplenism, my
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haemolysis, infection, hypersplenism, my elofibrosis, myelodysplasia, and aplastic
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elofibrosis, myelodysplasia, and aplastic
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anemia (2, 3). Pernicious anemia and SLE
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anemia (2, 3). Pernicious anemia and SLE
are both disorders of autoimmune etiology.
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are both disorders of autoimmune etiology.
Coexistence of SLE with other autoim
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Coexistence of SLE with other autoim mune disorders is common but association
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mune disorders is common but association of pernicious anemia and SLE is rare.
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of pernicious anemia and SLE is rare.
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n CASE REPORT
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CASE REPORT
A 27-year-old previously healthy woman
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A 27-year-old previously healthy woman was admitted with echymotic patches over
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was admitted with echymotic patches over
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are both disorders of autoimmune etiology.
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are both disorders of autoimmune etiology.
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the lower limbs. She was diagnosed with SLE with pernicious anemia and iron deficiency anemia. The rare as
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the lower limbs. She was diagnosed with SLE with pernicious anemia and iron deficiency anemia. The rare as sociation of SLE with pernicious anemia was reported previously in few patients. Treatment of SLE along with
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sociation of SLE with pernicious anemia was reported previously in few patients. Treatment of SLE along with supplementation is necessary for such patients. Since etiology for anemia in SLE can be of various kinds,
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supplementation is necessary for such patients. Since etiology for anemia in SLE can be of various kinds,
CASE REPORT
120 Reumatismo 3/2017
C.A. Mansoor, R. Narayan
CASE REPORT
136 mmol/l, potassium 3.8 mmol/l, AST 37 IU/l, ALT 39 IU/l, alkaline phosphatase 66 IU/l, total bilirubin 1.1 mg/dl, direct bilirubin 0.1 mg/dl, total protein 7.2 g/dl, albumin 4.4 g/dl, globulin 2.8 g/dl. Chest X Ray and electrocardiogram were normal.
Prothrombin time and partial thromboplas- tin times were normal. HIV, Hepatitis B and Hepatitis C serologies were negative.
Bone marrow aspirate showed hypercel- lular marrow with erythroid hyperplasia (micronormoblastic and megaloblastic maturation) (Figure 2A and B). Bone mar- row biopsy was hypercellular, showing erythroid hyperplasia with micro normo- blastic and megaloblastic maturation; the myeloid series showed normal maturation and differentiation, giant metamyelocytes, giant myelocytes and hypersegmanted neu- trophils; mild increase in megakaryocytes severe pallor and had multiple echymotic
patches over lower limbs. There were no other bleeding manifestations. Hemoglo- bin was 6.2 g/dl, total leucocyte count 4600/μl, platelet count 0.20x109/L, eryth- rocyte sedimentation rate 60 mm in 1 h and C- reactive protein was normal. The Hema- tocrit-corrected erythrocyte sedimentation rate was 24 mm/h.
In the peripheral smear, the RBCs showed moderate hypochromia, marked anisopoi- kilocytosis with microcytes, macrocytes, pencil shaped cells, macro ovalocytes, el- liptocytes, tear drop cells but no hemolysis (Figure 1); WBC’s were normal in number with few hypersegmented neutrophils and the platelet count was very low. Urinaly- sis was normal. Biochemical parameters showed random blood sugar 101mg%, urea19 mg/dl, creatinine 0.8 mg/dl, sodium
Figure 1 - A and B) Bone marrow aspirate showing hypercellular marrow with erythroid hy- perplasia (micronormoblastic and megaloblastic maturation); C and D) Bone marrow biopsy showing erythroid hyperplasia with micro normoblastic and megaloblastic maturation, hyper- segmented neutrophils and mild increase in megakaryocytes.
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and differentiation, giant metamyelocytes,
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and differentiation, giant metamyelocytes, giant myelocytes and hypersegmanted neu
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giant myelocytes and hypersegmanted neu trophils; mild increase in megakaryocytesuse
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row biopsy was hypercellular, showing
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row biopsy was hypercellular, showing erythroid hyperplasia with micro normo
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erythroid hyperplasia with micro normo blastic and megaloblastic maturation; the blastic and megaloblastic maturation; the
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myeloid series showed normal maturation
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myeloid series showed normal maturation and differentiation, giant metamyelocytes,
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and differentiation, giant metamyelocytes, giant myelocytes and hypersegmanted neu
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giant myelocytes and hypersegmanted neu
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A rare form of anemia in systemic lupus erythematosus CASE REPORT
with hypolobation (Figure 2C and D). Anti nuclear antibody was positive and anti-dou- ble-stranded DNA was strongly positive.
C3 and C4 levels were low (53 mg/dl and 8 mg/dl respectively). Serum vitamin B12 level was low (108 pg/mL) and folic acid level was normal. Intrinsic factor antibody was positive. Iron studies showed low fer- ritin (14.8 nano gram/ml), low serum iron (34 mcg/dL) and high total iron binding ca- pacity (540 mcg/dL).
A diagnosis of pernicious anemia and iron deficiency anemia complicating SLE was made based on the investigations. She was started on oral prednisolone 1 mg/kg, ste- roids, hydroxychloroquine and was also supplemented with hydroxycobalamin, folic acid and iron. When reviewed after three months, hemoglobin was 10.1 g/dl, total leucocyte count 4800/μl and platelet count 1.30x109/L.
n DISCUSSION AND CONCLUSIONS
SLE is a systemic autoimmune disease with variable multisystem involvement and het- erogeneous clinical features, ranging from mild to life-threatening. SLE can appear with hematological manifestations, alone or associated with features of involvement of other systems.
Common hematological abnormalities in SLE include leucopenia, lymphopenia, thrombocytopenia, autoimmune haemo- lytic anemia, thrombotic thrombocytope- nic purpura and myelofibrosis (2). Anemia of chronic disease is the most common form of anemia in SLE; auto-immune he- molytic anemia, iron deficiency anemia, drug-induced myelotoxicity, and anemia due to chronic renal failure are also often detected. Aplastic anemia, pure red cell aplasia, pernicious anemia, myelofibrosis, sideroblastic anemia, haemophagocytic syndrome, and thrombotic microangiopa- thy occurs less frequently (1).
Iron deficiency anemia (IDA) in SLE pa- tients can be caused by acute or chronic blood loss from the gastrointestinal tract, usually secondary to medications (nonste- roidal anti-inflammatory drugs or steroids),
or may be due to excessive menstrual bleed- ing. Our patient had IDA, mostly secondary to excessive menstrual bleeding. Bone mar- row biopsy specimens from patients with SLE having immune mediated bone marrow failure shows hypocellularity, morphological dysplasia, increased fibrosis and necrosis. T cell mediated inhibition of haemopoiesis is the major cause of bone marrow failure in SLE (1). Our patient had bone marrow hy- percellularity, erythroid hyperplasia with micro normoblastic and megaloblastic matu- ration along with low iron and vitamin B12, suggesting nutritional deficiency.
Pernicious anemia is a complex autoim- mune disease state caused by impaired ab- sorption of vitamin B12 due to an absence of the gastric glycoprotein, the intrinsic fac- tor (4). SLE can be associated with other au- toimmune diseases. The rare association of SLE with pernicious anemia was reported previously in few patients(5-8). Treatment of SLE along with B12 supplementation is necessary for such patients. Since etiology for anemia in SLE can be of various kinds, a detailed workup for identifying the under- lying mechanism is necessary.
n REFERENCES
1. Giannouli S, Voulgarelis M, Ziakas PD, Tzio- ufas AG. Anaemia in systemic lupus erythe- matosus: from pathophysiology to clinical as- sessment. Ann Rheum Dis. 2006; 65144-8.
2. Fayyaz A, Igoe A, Kurien BT, et al. Haema- tological manifestations of lupus. Lupus Sci Med. 2015; 2: e000078.
3. Keeling DM, Isenberg DA. Haematological manifestations of systemic lupus erythemato- sus. Blood Rev. 1993; 7: 199.
4. Osborne D, Sobczyńska-Malefora A. Autoim- mune mechanisms in pernicious anaemia and thyroid disease. Autoimmun Rev. 2015; 14:
763-8.
5. Benjilali L, Tazi-Mezalek Z, Harmouche H, et al. Pernicious anemia in a young man with systemic lupus erythematosus. Lupus. 2007;
16: 827-9.
6. Durand JM, Cretel E, Juhan V, et al. Systemic lupus erythematosus associated with pernicious anemia. Clin Exper Rheumatol. 1994; 12: 233.
7. Korbet SM, Corwin HL. Pernicious anemia associated with systemic lupus erythematosus.
J Rheumatol. 1986; 13: 193-4.
8. Singh A. An uncommon cause of anemia in systemic lupus erythematosus. Int J Rheum Dis. 2013; 16: 783-5.
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SLE with pernicious anemia was reported
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SLE with pernicious anemia was reported
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previously in few patients
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previously in few patients
of SLE along with B12 supplementation is
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of SLE along with B12 supplementation is necessary for such patients. Since etiology
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necessary for such patients. Since etiology for anemia in SLE can be of various kinds,
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for anemia in SLE can be of various kinds,
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erogeneous clinical features, ranging from
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erogeneous clinical features, ranging from mild to life-threatening. SLE can appear
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mild to life-threatening. SLE can appear with hematological manifestations, alone
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with hematological manifestations, alone or associated with features of involvement
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or associated with features of involvement Common hematological abnormalities in
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Common hematological abnormalities in SLE include leucopenia, lymphopenia,
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SLE include leucopenia, lymphopenia,
thrombocytopenia, autoimmune haemo
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thrombocytopenia, autoimmune haemo
lytic anemia, thrombotic thrombocytope
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lytic anemia, thrombotic thrombocytope
a detailed workup for identifying the under
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a detailed workup for identifying the under lying mechanism is necessary.
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lying mechanism is necessary.
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n
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of the gastric glycoprotein, the intrinsic fac
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of the gastric glycoprotein, the intrinsic fac tor (4). SLE can be associated with other au tor (4). SLE can be associated with other au
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toimmune diseases. The rare association of
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SLE with pernicious anemia was reported previously in few patients
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Pernicious anemia is a complex autoim-
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sorption of vitamin B12 due to an absence of the gastric glycoprotein, the intrinsic fac
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of the gastric glycoprotein, the intrinsic fac tor (4). SLE can be associated with other au
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tor (4). SLE can be associated with other au
