Search

Systemic anaplastic lymphoma kinase-negative (ALK-) anaplastic large cell lymphoma (ALCL) comprises a genomically heterogeneous disease that is considered a distinct entity by the 2016 World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Other than lymph nodes, systemic ALK- ALCL may affect extranodal tissues, sites where the inflammatory background may be especially prominent. In this scenario, myxoid change is exceptional in systemic ALK- ALCL. We describe a rare case of systemic ALK- ALCL with distinctive myxoid changes, carrying specific chromosomal aberrations that affect the clinical outcome. Careful morphological, immunohistochemical, and molecular workup is mandatory because a myxoid background should not be a reason to ignore the possibility of a lymphoma. Finally, extensive correlation with staging and the detection of prognostic biomarkers such as DUSP22 and TP63 rearrangements are essential for the diagnosis and prediction of clinical outcome in ALK- ALCL.

Germinotropic lymphoproliferative disorder is a rare and rather enigmatic novel entity with distinctive clinicopathological features, one of which is the typical co-infection by Human herpesvirus 8 and Epstein-Barr virus. Human herpesvirus 8 is a lymphotropic virus detected in Kaposi sarcoma, multicentric Castleman disease, primary effusion lymphoma, Human herpesvirus 8-positive diffuse large B cell lymphoma not otherwise specified, and germinotropic lymphoproliferative disorder. Co-infection by Human herpesvirus 8 and Epstein-Barr virus is identified only in two lymphoproliferative diseases: germinotropic lymphoproliferative disorder and primary effusion lymphoma, which are otherwise diseases with totally different clinical presentations and outcomes. Unlike primary effusion lymphoma mostly occurring in immunocompromised individuals and following an aggressive course, germinotropic lymphoproliferative disorder usually presents with single or multiple lymphadenopathy affecting mainly immunocompetent individuals and mostly follows an indolent course. Based on the PRISMA guidelines, we carried out a systematic search on PubMed/MEDLINE, Web of Science, Scopus, EMBASE, and Cochrane Library using the search terms "germinotropic" and "lymphoproliferative disorder." Current scientific literature reports just 19 cases of germinotropic lymphoproliferative disorder. The purpose of our systematic review is to improve our understanding of the disease, focusing on epidemiology, clinical presentation, pathological features, treatment, and outcome. In addition, we discuss the differential diagnosis with the other Human herpesvirus 8-related lymphoproliferative diseases as currently recognized in the World Health Organization classification, adding a focus on lymphoproliferative disorders showing overlapping features.

Summary Follicular lymphoma is a neoplasm derived from follicle center B cells, typically both centrocytes and centroblasts, in variable proportions according to the lymphoma grading. The pattern of growth may be entirely follicular, follicular and diffuse, and rarely completely diffuse. It represents the second most common non-Hodgkin lymphoma, after diffuse large B-cell lymphoma and is the most common low-grade mature B-cell lymphoma in western countries. In the majority of cases, follicular lymphoma is a nodal tumor, occurring in adults and frequently associated with the translocation t(14;18)(q32;q21)/IGH-BCL2. However, in recent years the spectrum of follicular lymphoma has expanded and small subsets of follicular lymphoma, which differ from common follicular lymphoma, have been identified and included in the current 2017 WHO classification. The aim of our review is to describe the broad spectrum of follicular lymphoma, pointing out that the identification of distinct clinicopathological variants of follicular lymphoma is relevant for patient outcomes and choice of treatment.

Paediatric-Type Nodal Follicular Lymphoma (PTNFL) is currently regarded as a distinct clinicopathological entity by the updated 4th edition of the WHO classification of haematopoietic tumours, harb...

Endosalpingiosis is a rare condition characterized by the presence of benign fallopian tubal-like glandular epithelium derived from Mullerian ducts, usually affecting the serosal surfaces of the pelvis and peritoneum. It is histologically differentiated from endometriosis as endosalpingiosis lacks endometrial stroma. Endosalpingiosis tends to affect older women and has been associated with ovarian serous tumors of low malignant potential. The extragenital endosalpingosis is typically without symptoms, reported only once as chronic pelvic pain. It rarely affects the appendix but can be mistaken for acute appendicitis or appendiceal tumors. No reports of endoscopic findings have been never described. Its treatment is challenging and provides a multidisciplinary approach with gynecologist, surgeon and gastrointestinal endoscopist. Our case reports for the first time an endoscopic finding of colonic salpingiosis and it is challenging both for the diagnosis and for the treatment.

Breakpoint cluster region - Abelson (BCR-ABL1) chimeric protein and mutated Nucleophosmin (NPM1) are often present in hematological cancers, but they rarely coexist in the same disease. Both anomalies are considered founder mutations that inhibit differentiation and apoptosis, but BCR-ABL1 could act as a secondary mutation conferring a proliferative advantage to a pre-neoplastic clone. The 2016 World Health Organization (WHO) classification lists the provisional acute myeloid leukemia (AML) with BCR-ABL1, which must be diagnosed differentially from the rare blast phase (BP) onset of chronic myeloid leukemia (CML), mainly because of the different therapeutic approach in the use of tyrosine kinase inhibitors (TKI). Here we review the BCR/ABL1 plus NPMc+ published cases since 1975 and describe a case from our institution in order to discuss the clinical and molecular features of this rare combination, and report the latest acquisition about an occurrence that could pertain either to the rare AML BCR-ABL1 positive or the even rarer CML-BP with mutated NPM1 at the onset. Differential diagnosis is based on careful analysis of genotypic and phenotypic features and anamnestic, clinical evolution, and background data. Therapeutic decisions must consider the broader clinical aspects, the comparatively mild effects of TKI therapy versus the great benefit that might bring to most of the patients, as may be incidentally demonstrated by our case history.

Anaplastic lymphoma kinase negative systemic anaplastic large cell lymphoma (ALK-ALCL) is a CD30+ T-cell malignant lymphoma which may involve both lymph nodes and extranodal tissues, showing important clinical differences from ALK-positive ALCL (ALK + ALCL). ALK- ALCL is considered a specific entity by the 2016 World Health Organization (WHO) classification of hematolymphoid neoplasms.We describe an exceptional case of ALK- ALCL with a striking “Hodgkin-like” cytomorphology and a very uncommon nuclear expression of PAX5.

In Hodgkin Lymphoma (HL), about 20% of patients still have relapsed/refractory disease and late toxic effects rate continue to rise with time. 'Early FDG-PET' and tissue macrophage infiltration (TAM) emerged as powerful prognostic predictors. The primary endpoint was to investigate the prognostic role of both early FDG-PET and TAM; the secondary endpoint was to test if early FDG-PET positivity could correlate with high TAM score. A cohort of 200 HL patients was analysed. Induction treatment plan consisted of two to six courses of ABVD and, if indicated, involved field radiation therapy. All patients repeated CT scan and FDG-PET after two cycles and after the completion of therapy. TAM in diagnostic specimens was determined by immunohistochemistry with a monoclonal antibody (anti-CD68 KP1). Overall, early FDG-PET was negative in 163 patients (81.5%) and positive in 37 patients (18.5%), showing a significant correlation with the achievement of CR (p

Follicular dendritic cell sarcoma (FDCS) is an uncommon tumor arising from follicular dendritic cells (FDCs) in the lymph nodes or in extranodal sites. We herein report an unusual case of a 30-year-old man who presented with a FDCS of the omentum and massive liver involvement. The tumor proliferation was a diffuse growth composed by large and highly pleomorphic cells. On immunostaining, B and T cell markers; CD30, myeloid, and histiocytic markers; and S100 were negative whereas CD21, CD23, CD35, clusterin, and fascin were positive. Unexpectedly, large spectrum cytokeratin clone AE1/AE3 was focally and variably expressed. Despite the latter atypical finding and the misleading morphology, immunohistochemistry allowed for a conclusive diagnosis of FDCS.

Primary induction failure (PIF) in patients with acute myeloid leukemia (AML) is defined by the persistence of more than 5% of blasts [1] after 1–2 cycles of intensive chemotherapy (ICT) [1,2]. PIF...

Objectives This study was undertaken to define and compare geographic coronary artery inflammation in patients who were dying of acute myocardial infarction (AMI), chronic stable angina (SA), and noncardiac causes (CTRL). Background Biochemical markers and flow cytometry provide indirect evidence of diffuse coronary inflammation in patients dying of acute coronary syndromes. Yet no histopathologic studies have corroborated these findings. A key unanswered question is whether the inflammatory burden involves the entire coronary tree or is limited to a few plaques. Methods We examined 544 coronary artery segments from 16 patients with AMI, 109 segments from 5 patients with SA, and 304 coronary segments from 9 patients with CTRL. Results An average of 6.8 ± 0.5 vulnerable segments per patient were found in the AMI group (in addition to culprit lesions) compared with an average of 0.8 ± 0.3 and 1.4 ± 0.3 vulnerable lesions/patient in the SA and CTRL groups, respectively. The AMI group, independent of the type of plaque observed, showed significantly more inflammatory infiltrates compared with the SA and CTRL groups (121.6 ± 12.4 cell × mm2vs. 37.3 ± 11.9 cell × mm2vs. 26.6 ± 6.8 cell × mm2, p = 0.0001). In AMI patients, active inflammation was not only evident within the culprit lesion and vulnerable plaques but also involved stable plaques. These showed a three- to four-fold higher inflammation than vulnerable and stable plaques from the SA and CTRL groups, respectively. Conclusions This histopathologic study found that both vulnerable and stable coronary plaques of patients dying of AMI are diffusely infiltrated by inflammatory cells.

TO THE EDITOR: The treatment approach to acute myeloid leukemia (AML) transformed from high-risk myelodysplastic syndrome (MDS) after azacitidine failure is not standardized and the clinical results achievable in this setting are generally disappointing [1,2,3]. Similarly, poor outcomes have also been reported in the setting of AML transformation following azacitidine discontinuation due to reasons other than hematologic progression in MDS patients initially responding to hypomethylating therapy [4]. Little is known of azacitidine-responsive MDS patients who progressed to AML with 20-30% bone marrow (BM) blasts after suspension of this agent, nor of the possibility to re-induce hematological control after the resumption of hypomethylating therapy.

TO THE EDITOR: The occurrence of monoclonal gammopathy of undetermined significance (MGUS) and essential thrombocytopenia (ET) in the same patient is quite rare. With an anecdotal purpose, we herein report the long-term clinical history of a patient who presented with simultaneous evolution to multiple myeloma (MM) and to acute biphenotypic leukemia from MGUS and ET, respectively, with the latter conditions simultaneously diagnosed 37 years prior to this case.

The case of an elderly and frail patient affected by simultaneous large B-cell non-Hodgkin lymphoma and acute myeloid leukaemia is reported. The complete remissions of both diseases by azacitidine and rituximab–bendamustine regimen were achieved.

TO THE EDITOR: Transformation from myelodysplastic syndrome (MDS) to chronic myelomonocytic leukemia (CMML) is rarely observed. However, this has been reported in cases of refractory anemia with ring sideroblasts or excess of blasts [1-4]. Moreover, MDS patients may present with monocytosis that does not meet the diagnostic criteria of CMML, which makes diagnosis and classification of these atypical mixed disorders a challenge [5, 6]. These difficulties in diagnostic classification and prognostic stratification may be concerning with regard to decision-making, particularly in this new era of effective disease-modifying therapies, such as hypomethylating agents [6, 7]. Recently, we faced such concerns during the management of a patient who developed CMML 7 years after having been diagnosed with refractory anemia (RA). The full clinical onset of CMML was preceded by progressive loss of response to ongoing treatment with an erythropoiesis-stimulating agent (ESA), worsening anemia, thrombocytopenia, leukocytosis, and increasing monocytosis. A morphological study of the peripheral blood (PB) and bone marrow (BM) revealed the coexistence of myelodysplastic and myeloproliferative syndromes. A cytogenetic alteration (45, X0,-Y), which was not present at diagnosis of RA 7 years earlier, was also detected during the CMML phase. The patient received azacitidine and showed a good response. Here, we describe this rare case and its implications in disease classification and management. On January 2005, a 74-year-old man presented with moderately macrocytic slight thrombocytopenia. Five years earlier (in 2000), he had received postoperative radiotherapy after radical prostatectomy for prostate cancer. Apart from this prostatic neoplasm, for which a regular oncological follow-up had confirmed a persistent complete remission until then, he mentioned one previously cured gastric ulcer and well-controlled hypertension. He complained of fatigue and general unease for the past few weeks. A complete blood count prescribed by his general practitioner had revealed macrocytic anemia with a low reticulocyte count, mild thrombocytopenia, and slight neutropenia. On admission, he appeared pale and fatigued. A comprehensive laboratory evaluation did not reveal any remarkable abnormalities. His coagulative profile and renal and hepatic function were normal. Suspicions of hemolytic disorders, virus infections, and iron and vitamin deficiencies were also dismissed. Morphological examination of PB smears showed isolated erythrocyte macrocytosis but did not provide any other diagnostic findings. BM aspiration and trephine biopsy were performed. BM examination revealed hypercellular BM with evident erythrodysplasia and 6% of blasts without fibrosis. Conventional karyotyping and fluorescence in situ hybridization analyses did not show abnormalities. Therefore, the patient was diagnosed with MDS, RA, according to the French-American-British classification. The patient had an International Prognostic Scoring System score of 1 (intermediate-1 risk). On admission and during the diagnostic phase, he received 4 units of red blood cell (RBC) concentrates. Thus, the patient was scheduled to receive ESA at a weekly dose of 40,000 units by subcutaneous injection (in January 2005). Since then, the patient was regularly followed-up at our clinic and continued to receive erythropoietin treatment. Normal PB values were maintained without the requirement for transfusion, clinical complications, or side effects until June 2012, when his hematologic status slowly began to deteriorate. Worsening anemia (requiring RBC transfusions), thrombocytopenia, and leukocytosis with absolute monocytosis became prominent. A comprehensive medical examination was performed. The examination of PB smears showed absolute monocitosis with 2% of circulating blasts. BM examination showed marked erythroid dysplasia and monocytosis with 15% blasts. A karyotype abnormality, such as 45, X0,-Y, which was not detected at initial diagnosis of RA 7 years earlier, was also found; however, the JAK2 V617F mutation was absent. Therefore, the patient was diagnosed with RA, coexisting with type II CMML. The patient had a MD Anderson Prognostic Score of 3 (intermediate-2 risk) [8]. After a brief course of hydroxycarbamide, administered in order to reduce leukocytosis, the patient received 6 cycles of azacitidine (75 mg/m2, schedule 5+2+2, each cycle every 4 weeks), according to approved indications [9]. After the second course, transfusion independence was achieved and the PB counts significantly improved with disappearance of monocytosis. Complete remission of CMML was achieved after 6 courses of hypomethylating therapy, without any adverse events. In conclusion, this case represents an atypical presentation of CMML secondary to MDS. In addition to its rarity and anecdotal interest, we believe that this report should be discussed with regard to several topics, such as the possible evolution of low-risk MDS in CMML, the existence of secondary CMML as a distinct disease, its absence in the current classification systems, and, finally, the efficacy of hypomethylating therapy [4, 10].

Immune thrombocytopoenia (ITP) is one of the most common autoimmune manifestations of B cell lymphoproliferative diseases. The association with multiple myeloma (MM) and solid tumours is rare. Here, a case of ITP associated with asymptomatic multiple myeloma and colon carcinoma, refractory to standard therapy and responsive to rituximab, is described. ITP should be considered in the differential diagnosis of thrombocytopoenia in MM and colon cancer. Understanding of the potential risk and reversibility of ITP should aid in the management of these patients.

Reduced-intensity conditioning regimens have reshaped the clinical presentation of graft-versus-host disease after hematopoietic stem cell transplants. However, histopathologic features of graft-versus-host disease following reduced-intensity conditioning regimens have not been fully characterized. In a series of 112 biopsies (skin, n = 60; gastrointestinal [GI] tract, n = 44; liver, n = 8), we described the morphologic profile of graft-versus-host disease following reduced-intensity conditioning and investigated whether histopathologic changes of graft-versus-host disease following reduced-intensity conditioning have a diagnostic and/or prognostic value. Forty-four patients (49.5%) experienced acute graft-versus-host disease, 2(2.2%) late-onset acute graft-versus-host disease (grade I, n = 13; grade II-IV, n = 33), 24(27.0%) chronic graft-versus-host disease (de novo n = 12, progressive n = 12) and 19 (21.3%) overlap syndrome. In the skin, we observed: (i) phase-nonspecific changes, such as acute graft-versus-host disease features in chronic graft-versus-host disease patients (n = 4/24; 16.6%), (ii) subtle alterations such as superficial fibrosis in widened dermal papillae (n = 8), in acute graft-versus-host disease/late-onset graft-versus-host disease (n = 6/46; 13.0%) or chronic graft-versus-host disease (n = 2/24, 8.3%) patients, and (iii) features of chronic and acute graft-versus-host disease coexisting in the same specimen in overlap syndrome (n = 3/19; 15.7%). In the GI tract, we did not demonstrate peculiar features differing from those commonly observed in the myeloablative setting. By univariate analysis, a reduced overall survival was associated with graft-versus-host disease type (chronic graft-versus-host disease P = .006, acute graft-versus-host disease P = .03), older age (P = .04), and histopathologic diagnosis of "consistent with" + definite graft-versus-host disease (P = .02). Histopathologic diagnosis retained an independent prognostic value by multivariate analysis (P = .01). The present study indicates that pathologists should be aware of the peculiar morphologic changes of cutaneous graft-versus-host disease following reduced-intensity conditioning and further recommends histopathology in the diagnostic workup of graft-versus-host disease in patients undergoing reduced-intensity conditioning regimen. (C) 2011 Elsevier Inc. All rights reserved.

In the course of follicular lymphomas (FL), although a disease transformation in clinically more aggressive lymphoproliferative disorders, mostly diffuse large B-cells non-Hodgkin lymphomas (NHL), ...

BackgroundExtraskeletal cartilaginous tumors are uncommon. Osteochondromas usually arise from the metaphyseal region of the growing skeleton.Case presentationA 53 year old man presented with a three years history of anterior knee pain and inability to flex his knee more than 90°. Clinical examination and imaging studies revealed a nodular calcific mass in the anterior portion of the knee, displacing the medial portion of the patellar tendon. Following excision, histopathology confirmed the diagnosis of extra-osseous osteochondroma-like soft tissue mass, with no recurrence 24 months after surgery.ConclusionAn integrated clinical-pathologic diagnosis helps to clarify the nature of extraskeletal cartilaginous tumors that can arise at unusual anatomic site. Complete local surgical excision is the management of choice.

A 46-year-old female presented a six-month history of posterior heel pain. Clinical and radiographical examination revealed a nodular calcified mass into the subcutaneous tissue of the Achilles tendon bursa. Following excision, histopathology showed an extraskeletal soft-tissue chondroma. Follow up at 24 months showed no recurrence. To our knowledge, this is the first description of a soft tissue chondroma at this site: some soft tissue tumors develop at unusual anatomic location.

This study was undertaken to define and compare geographic coronary artery inflammation in patients who were dying of acute myocardial infarction (AMI), chronic stable angina (SA), and noncardiac causes (CTRL).Biochemical markers and flow cytometry provide indirect evidence of diffuse coronary inflammation in patients dying of acute coronary syndromes. Yet no histopathologic studies have corroborated these findings. A key unanswered question is whether the inflammatory burden involves the entire coronary tree or is limited to a few plaques.We examined 544 coronary artery segments from 16 patients with AMI, 109 segments from 5 patients with SA, and 304 coronary segments from 9 patients with CTRL.An average of 6.8 +/- 0.5 vulnerable segments per patient were found in the AMI group (in addition to culprit lesions) compared with an average of 0.8 +/- 0.3 and 1.4 +/- 0.3 vulnerable lesions/patient in the SA and CTRL groups, respectively. The AMI group, independent of the type of plaque observed, showed significantly more inflammatory infiltrates compared with the SA and CTRL groups (121.6 +/- 12.4 cell x mm2 vs. 37.3 +/- 11.9 cell x mm2 vs. 26.6 +/- 6.8 cell x mm2, p = 0.0001). In AMI patients, active inflammation was not only evident within the culprit lesion and vulnerable plaques but also involved stable plaques. These showed a three- to four-fold higher inflammation than vulnerable and stable plaques from the SA and CTRL groups, respectively.This histopathologic study found that both vulnerable and stable coronary plaques of patients dying of AMI are diffusely infiltrated by inflammatory cells.

To the Editor, Myeloid sarcoma (MS) of the gingiva is an extremely rare observation in the context of different myeloproliferative disorders [1–3], including chronic myelomonocytic leukemia (CMML) [1, 4] and acute myeloid leukemia (AML). In CMML patients, gingival hypertrophy is often associated with tumor progression and a more aggressive disease [4]. Although the evolution of myelodysplastic syndrome (MDS) in overt AML is a common occurrence, isolated gingival MS in patients with stable MDS without AML progression has been only exceptionally observed [3]. Herein, we report on a case of gingival MS occurred as isolated AML progression in a previously diagnosed MDS. The patient was a 63year-old Caucasian male who was diagnosed as having MDS, subtype refractory cytopenia with multilineage dysplasia; he presented with trilinear cytopenia (transfusion-dependent severe anemia, thrombocytopenia, and neutropenia), bone marrow (BM) hypocellularity with 3 % of blasts and a cytogenetic abnormality consistent with trisomy 8. According to the IPSS [5] and WPSS [6], the MDS risk was classified as Int-1 and intermediate categories, respectively. The patient had always been healthy and had no significant comorbidities; in particular, no oral and/or gastrointestinal diseases were reported. Given the immune-mediated pathogenesis likely involved in most forms of hypoplastic MDS [7], the patient was managed by cyclosporine. This treatment effort resulted in a substantially maintained stable disease without any significant toxicity. Six months after the diagnosis, the patient complained a well-circumscribed little enlargement of the right upper gingiva surrounded by a painful ulcer. An excision biopsy of the intraoral mass was performed. Histological analysis showed submucosal infiltration by aggregates of myeloperoxidase and CD68 positive myeloid blast cells. A comprehensive hematological revaluation, including BM aspirate and trephine biopsy and a karyotype analysis, revealed a stable MDS without any evidence of AML progression in the BM; in particular, the percentage of blast cells was the same of that found at the MDS diagnosis. Cytogenetic analysis confirmed the presence of trisomy 8. Two courses of fludarabine and high dose of cytosine arabinoside regimen were given resulting in the disappearance of the gingival swelling and the BM blast cells. A suitable donor was not available, for which autologous stem cell transplantation (ASCT) with busulfan and cyclophosfamide conditioning regimen was given as consolidation treatment. However, 3 months after ASCT, he presented a relapse of AML, without any extramedullary localization. AML rapidly progressed until the patient’s death. The unusual intraoral localization of MS and the presence of the trisomy 8 in our case, may suggest a possible link between these findings. Indeed, recurrent oral ulcers and the excess of chromosome The authors have no affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. No writing assistance was utilized in the production of this manuscript. The authors declare that they have full control of all primary data and they agree to allow the journal to review their data if requested. A. Tendas : L. Scaramucci :M. Giovannini : L. Cupelli : A. Perrotti : P. de Fabritiis Hematology Unit, S. Eugenio Hospital, Rome, Italy

TO THE EDITOR: Gelatinous bone marrow transformation (GMT) is a rare bone marrow (BM) disorder of unknown pathogenesis. It is characterized by fat cell atrophy, focal loss of hematopoietic cells, and deposition of extracellular gelatinous substances (mucopolysaccharides rich in hyaluronic acid) [1]. The pathogenesis of GMT involves the deposition of hyaluronic acid, which hampers hematopoiesis by altering the BM microenvironment and stroma and disruption of the interactions between BM cells and cell signaling molecules [1]. GMT has been reported in association with chronic debilitating diseases, such as anorexia nervosa, malnutrition, and human immunodeficiency virus (HIV) infection, and after cytotoxic treatments [2]; in addition, GMT has been described in patients with myelodysplastic syndrome [3], acute myeloblastic leukemia [3], and idiopathic myelofibrosis [4]. However, idiopathic GMT has also been reported [5]. Herein, we report a case of idiopathic GMT in a patient who had an unusual clinical course and long-term survival. The patient was a 64-year-old Caribbean woman who had been living in Rome for more than 30 years. She was referred to our clinic in December 2005 because of anemia. Her past medical history was unremarkable. She did not use alcohol, drugs, or tobacco, and her nutritional status was excellent. Physical examination was unremarkable with the exception of pallor; the patient did not have either spleen or liver enlargement. A complete blood count at admission showed normochromic-normocytic anemia with reticulocytopenia and normal white blood cell and platelet counts. Total and unconjugated bilirubin and serum haptoglobin levels were normal. Direct and indirect indirect Coombs tests were negative. Serological tests for HIV, cytomegalovirus, Epstein-Barr virus, parvovirus B19, and hepatitis B and C viruses were negative. In addition, serum levels of tri-iodothyronine were normal. Moreover, no clinical or laboratory features of autoimmune disease were detected. Examination of peripheral blood smears revealed normal RBC; erythrocyte fragments were not detectable. Radiological examinations, which included a whole-body computed tomography scan, revealed no abnormalities. Occult blood loss was ruled out by fecal and urine analyses. A BM aspirate performed at admission resulted in a dry tap. Therefore, a BM trephine biopsy was taken. Histological examination of the BM sample revealed GMT (Fig. 1). The patient did not respond to any potentially disease-modifying treatment, including erythropoietin, prednisone, and cyclosporine. Therefore, she was managed with supportive measures, primarily transfusions. The clinical course was uncomplicated and her hematological status has remained stable. To date, 80 months after the diagnosis, she is managed only with supportive measures, consisting of 2 units of packed RBC every 3 weeks, along with iron chelation therapy. In addition, her BM features, which have been evaluated annually by BM trephine biopsies, have remained stable. In conclusion, we have described a case of an apparently healthy woman who presented with GMT approximately 7 years ago, in whom anemia was the only clinical manifestation of hematopoietic impairment. To the best of our knowledge, no such case of GMT has been reported previously as an idiopathic primary condition. Fig. 1 Serous atrophy in a diffuse and severe hypocellular background (A). Reduced fat cells, a diffuse extracellular gelatinous amorphous material, small typical lymphocytes, and mature plasma cells were also observed (B). Small islets of hematopoietic cells ...