Contributions
Abstract: PB2300
Type: Publication Only
Background
Autoimmune myelofibrosis (AIMF) is a rare cause of marrow fibrosis. It has been reported in association with autoimmune disorders, like systemic lupus erythematosus among others (Vergara-Lluiri et al 2014). Our study presents two cases of common variable immunodeficiency (CVID) associated AIMF and one case of relapsing-remitting multiple sclerosis (RRMS) associated AIMF. Autoimmunity in the context of CVID occur in 20–30% of patients being cytopenias the most common autoimmune condition (Xiao et al. 2014).
Aims
To review the incidence and evolution of patients diagnosed with AIMF in our center.
Methods
We retrospectively reviewed patients from the Pathology service data base and medical histories.
Results
From 2000 to 2018, 34 patients were newly diagnosed of MF. Three (8.8%) were AIMF: two women (66%) diagnosed with CVID and one male (33%) diagnosed with RRMS. Median age at diagnosis was 38. One patient (33%) died due to an infection. Patients evolution and pathology (Table 1). Case A: A 25-year-old female diagnosed with CVID in 2010 in the setting of severe bicytopenia (600 neutrophils/uL and 6.000 platelets/uL), and mild/moderate infections. Despite autoimmunity tests were negative, direct coombs test was positive and has remained so ever since. Bone marrow biopsy (BMB) was compatible with AIMF. Immunoglobulins (Ig) 0,5g/Kg/21 days were given with CBC normalization. Corticoids were added and slowly tapered. Interestingly, while before each cycle platelets dropped under 10.000/uL, neutrophils sustainably normalized. Eltrombopag was iniciated in April/2011 with good results (>50.000 platelets/uL pre-Ig). Six years later, pre-Ig platelets account dropped to <10.000/uL. We switched to Romiplostim (raised to 4mcg/kg in the last visit) and increased Ig to 1g/kg with encouraging results (11.000 platelets/uL pre-Ig in the last visit). Case B: A 43-year-old female diagnosed in 2003 CVID and receiving Ig 0,5g/kg/21 days and corticosteroids, was sent to our clinic in 2010 due to grade IV neutropenia and severe infections. Neutrophils oscillated between 300/uL and 1000/uL and platelets progressively dropped to 25-30.000/uL. The patient presented with positive antibodies anti-parietal cell (1/160), vitamin B12 in normal range. BMB was compatible with AIMF. In August/2017 due to grade IV bicytopenia, Ig were increased to every 14 days and mycophenolate was started maintaining neutrophils >600/uL and platelets >40.000/uL. Case C: A 38-year-old male diagnosed with RRME in 2008, last treatment alemtuzumab (June/2016) presented with asymptomatic grade IV thrombocytopenia in May/2017. Dexamethasone 40mg for 4 days (2 cycles) was started raising platelets to 253.000/uL. However, within a month, he presented with severe pancytopenia. Positive anti-neutrophil cytoplasmic antibodies were detected. BMB was compatible with AIMF. He received rituximab (2 doses) and Ig 1g/Kg/2days with no response. In September/2017 erythropoietin and plasma exchange was initiated with normalization of the platelet account (1 plasma volume was replaced with 0.5% human albumin diary (x4), then once a week). Neutrophils oscillated between 20 and 2.000/uL (good response to G-CSF and plasma exchange intensification). On February/2018 he presented with sepsis (gripeA) and died in the intensive care unit.
Conclusion
AIMF is a rare disease of which we present previously unreported cases associated with CVID and RRMS. Thrombopoietin agonists can be considered as a therapeutic option for these patients.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Autoimmunity, Myelofibrosis, Thrombopoietin (TPO)
Abstract: PB2300
Type: Publication Only
Background
Autoimmune myelofibrosis (AIMF) is a rare cause of marrow fibrosis. It has been reported in association with autoimmune disorders, like systemic lupus erythematosus among others (Vergara-Lluiri et al 2014). Our study presents two cases of common variable immunodeficiency (CVID) associated AIMF and one case of relapsing-remitting multiple sclerosis (RRMS) associated AIMF. Autoimmunity in the context of CVID occur in 20–30% of patients being cytopenias the most common autoimmune condition (Xiao et al. 2014).
Aims
To review the incidence and evolution of patients diagnosed with AIMF in our center.
Methods
We retrospectively reviewed patients from the Pathology service data base and medical histories.
Results
From 2000 to 2018, 34 patients were newly diagnosed of MF. Three (8.8%) were AIMF: two women (66%) diagnosed with CVID and one male (33%) diagnosed with RRMS. Median age at diagnosis was 38. One patient (33%) died due to an infection. Patients evolution and pathology (Table 1). Case A: A 25-year-old female diagnosed with CVID in 2010 in the setting of severe bicytopenia (600 neutrophils/uL and 6.000 platelets/uL), and mild/moderate infections. Despite autoimmunity tests were negative, direct coombs test was positive and has remained so ever since. Bone marrow biopsy (BMB) was compatible with AIMF. Immunoglobulins (Ig) 0,5g/Kg/21 days were given with CBC normalization. Corticoids were added and slowly tapered. Interestingly, while before each cycle platelets dropped under 10.000/uL, neutrophils sustainably normalized. Eltrombopag was iniciated in April/2011 with good results (>50.000 platelets/uL pre-Ig). Six years later, pre-Ig platelets account dropped to <10.000/uL. We switched to Romiplostim (raised to 4mcg/kg in the last visit) and increased Ig to 1g/kg with encouraging results (11.000 platelets/uL pre-Ig in the last visit). Case B: A 43-year-old female diagnosed in 2003 CVID and receiving Ig 0,5g/kg/21 days and corticosteroids, was sent to our clinic in 2010 due to grade IV neutropenia and severe infections. Neutrophils oscillated between 300/uL and 1000/uL and platelets progressively dropped to 25-30.000/uL. The patient presented with positive antibodies anti-parietal cell (1/160), vitamin B12 in normal range. BMB was compatible with AIMF. In August/2017 due to grade IV bicytopenia, Ig were increased to every 14 days and mycophenolate was started maintaining neutrophils >600/uL and platelets >40.000/uL. Case C: A 38-year-old male diagnosed with RRME in 2008, last treatment alemtuzumab (June/2016) presented with asymptomatic grade IV thrombocytopenia in May/2017. Dexamethasone 40mg for 4 days (2 cycles) was started raising platelets to 253.000/uL. However, within a month, he presented with severe pancytopenia. Positive anti-neutrophil cytoplasmic antibodies were detected. BMB was compatible with AIMF. He received rituximab (2 doses) and Ig 1g/Kg/2days with no response. In September/2017 erythropoietin and plasma exchange was initiated with normalization of the platelet account (1 plasma volume was replaced with 0.5% human albumin diary (x4), then once a week). Neutrophils oscillated between 20 and 2.000/uL (good response to G-CSF and plasma exchange intensification). On February/2018 he presented with sepsis (gripeA) and died in the intensive care unit.
Conclusion
AIMF is a rare disease of which we present previously unreported cases associated with CVID and RRMS. Thrombopoietin agonists can be considered as a therapeutic option for these patients.
Session topic: 16. Myeloproliferative neoplasms - Clinical
Keyword(s): Autoimmunity, Myelofibrosis, Thrombopoietin (TPO)