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Hemophagocytic lymphohistiocytosis in a patient with Goodpasture's syndrome: a rare clinical association.

Basnet A, Cholankeril MR - Am J Case Rep (2014)

Bottom Line: This was initially attributed to the immunosuppressive effect of hydroxychloroquine.Here, we present a rare case of Goodpasture's syndrome with overlap and pauci-immune vasculitis, which may have triggered the HLH.This correlation has not been described before in the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Trinitas Regional Medical Center, Seton Hall University of Graduate Medical Education, Elizabeth, USA.

ABSTRACT

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome. HLH can occur in the setting of an autoimmune disease, chronic immunosuppression, malignancy, and infection. We discuss a rare case of a young woman who was diagnosed with Goodpasture's syndrome that was most likely complicated by HLH. To the best of our knowledge, this is the first report of HLH in the setting of this rare autoimmune disease.

Case report: A 31-year-old woman who was diagnosed with Goodpasture's syndrome 7 years prior presented with febrile neutropenia. She was initially receiving treatment with azathioprine and prednisone, which was subsequently switched to hydroxychloroquine. Over time, she had developed polyarthritis and was later diagnosed with MPO-ANCA-positive vasculitides. On this admission, her clinical status deteriorated from persistent pancytopenia. This was initially attributed to the immunosuppressive effect of hydroxychloroquine. A bone marrow biopsy was performed and revealed hypercellular bone marrow without any cytogenetic abnormalities. Due to a prolonged pancytopenia thought to be of autoimmune etiology, treatment with high-dose steroids was initiated. With the persistent febrile episodes, hepatosplenomegaly on examination, and laboratory workup that revealed hyperferritinemia and pancytopenia, HLH syndrome was suspected. A repeat bone marrow biopsy confirmed this diagnosis with the presence of hemophagocytosis, demonstrated by the presence of histiocytes engulfing erythroid cells. She also met 5 of 8 diagnostic criteria, which confirmed the diagnosis of HLH. The patient eventually died despite aggressive treatment with high-dose steroid therapy for her autoimmune disorder, as well intravenous antibiotics and supportive care for her underlying infections.

Conclusions: HLH is a syndrome marked by a hyper-inflammatory state aggravated by specific triggers. To make the diagnosis of HLH, at least 5 of the 8 criteria must be met. Treatment involves suppression of the overwhelming inflammatory response by the use of immunomodulators. The mortality rate can range from 50-90% due to delayed recognition and onset of treatment. Here, we present a rare case of Goodpasture's syndrome with overlap and pauci-immune vasculitis, which may have triggered the HLH. This correlation has not been described before in the literature.

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Immunofluroscence demonstrating diffuse, linear staining (red arrow) of glomerular basement membrane for IgG and C3, suggesting anti-GBM antibody deposition in renal biopsy.
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f2-amjcaserep-15-431: Immunofluroscence demonstrating diffuse, linear staining (red arrow) of glomerular basement membrane for IgG and C3, suggesting anti-GBM antibody deposition in renal biopsy.

Mentions: We present the case of a 31-year-old woman who was well known to our institution for the last 7 years. She originally had a medical history that was significant for hypertension. Within months of her original diagnosis of hypertension, she was admitted to the hospital with complaints of hemoptysis. Chest X-ray done at that time revealed extensive bilateral perihilar consolidations. Sputum culture had been performed, which stained negative for acid-fast bacilli (AFB). Upon further laboratory evaluation, she was found to be in acute renal failure. Due to the complaints of hemoptysis seen in the setting of renal failure, a renal biopsy had been performed. Immunofluorescence microscopy had demonstrated a diffuse, necrotizing, crescentic, pauci-immune type glomerulonephritis. A linear deposition of IgG along the glomerular basement membrane, suggestive of anti-GBM nephritis, was seen (Figures 1 and 2), but serum anti-GBM antibodies were negative. With this significant biopsy result, coupled with the presentation of hemoptysis from pulmonary hemorrhage, the diagnosis of Goodpasture’s syndrome was made.


Hemophagocytic lymphohistiocytosis in a patient with Goodpasture's syndrome: a rare clinical association.

Basnet A, Cholankeril MR - Am J Case Rep (2014)

Immunofluroscence demonstrating diffuse, linear staining (red arrow) of glomerular basement membrane for IgG and C3, suggesting anti-GBM antibody deposition in renal biopsy.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4199464&req=5

f2-amjcaserep-15-431: Immunofluroscence demonstrating diffuse, linear staining (red arrow) of glomerular basement membrane for IgG and C3, suggesting anti-GBM antibody deposition in renal biopsy.
Mentions: We present the case of a 31-year-old woman who was well known to our institution for the last 7 years. She originally had a medical history that was significant for hypertension. Within months of her original diagnosis of hypertension, she was admitted to the hospital with complaints of hemoptysis. Chest X-ray done at that time revealed extensive bilateral perihilar consolidations. Sputum culture had been performed, which stained negative for acid-fast bacilli (AFB). Upon further laboratory evaluation, she was found to be in acute renal failure. Due to the complaints of hemoptysis seen in the setting of renal failure, a renal biopsy had been performed. Immunofluorescence microscopy had demonstrated a diffuse, necrotizing, crescentic, pauci-immune type glomerulonephritis. A linear deposition of IgG along the glomerular basement membrane, suggestive of anti-GBM nephritis, was seen (Figures 1 and 2), but serum anti-GBM antibodies were negative. With this significant biopsy result, coupled with the presentation of hemoptysis from pulmonary hemorrhage, the diagnosis of Goodpasture’s syndrome was made.

Bottom Line: This was initially attributed to the immunosuppressive effect of hydroxychloroquine.Here, we present a rare case of Goodpasture's syndrome with overlap and pauci-immune vasculitis, which may have triggered the HLH.This correlation has not been described before in the literature.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Trinitas Regional Medical Center, Seton Hall University of Graduate Medical Education, Elizabeth, USA.

ABSTRACT

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome. HLH can occur in the setting of an autoimmune disease, chronic immunosuppression, malignancy, and infection. We discuss a rare case of a young woman who was diagnosed with Goodpasture's syndrome that was most likely complicated by HLH. To the best of our knowledge, this is the first report of HLH in the setting of this rare autoimmune disease.

Case report: A 31-year-old woman who was diagnosed with Goodpasture's syndrome 7 years prior presented with febrile neutropenia. She was initially receiving treatment with azathioprine and prednisone, which was subsequently switched to hydroxychloroquine. Over time, she had developed polyarthritis and was later diagnosed with MPO-ANCA-positive vasculitides. On this admission, her clinical status deteriorated from persistent pancytopenia. This was initially attributed to the immunosuppressive effect of hydroxychloroquine. A bone marrow biopsy was performed and revealed hypercellular bone marrow without any cytogenetic abnormalities. Due to a prolonged pancytopenia thought to be of autoimmune etiology, treatment with high-dose steroids was initiated. With the persistent febrile episodes, hepatosplenomegaly on examination, and laboratory workup that revealed hyperferritinemia and pancytopenia, HLH syndrome was suspected. A repeat bone marrow biopsy confirmed this diagnosis with the presence of hemophagocytosis, demonstrated by the presence of histiocytes engulfing erythroid cells. She also met 5 of 8 diagnostic criteria, which confirmed the diagnosis of HLH. The patient eventually died despite aggressive treatment with high-dose steroid therapy for her autoimmune disorder, as well intravenous antibiotics and supportive care for her underlying infections.

Conclusions: HLH is a syndrome marked by a hyper-inflammatory state aggravated by specific triggers. To make the diagnosis of HLH, at least 5 of the 8 criteria must be met. Treatment involves suppression of the overwhelming inflammatory response by the use of immunomodulators. The mortality rate can range from 50-90% due to delayed recognition and onset of treatment. Here, we present a rare case of Goodpasture's syndrome with overlap and pauci-immune vasculitis, which may have triggered the HLH. This correlation has not been described before in the literature.

Show MeSH
Related in: MedlinePlus