Monoartritis del codo e infarto óseo como presentación inicial de enfermedad linfoproliferativa / Elbow monoarthritis and bone infarction as a form of presentation of B cell lymphoma: case report

We report a 68-year-old woman presenting with pain and swelling in her left elbow. An elbow magnetic resonance with gadolinium evidenced bone marrow infiltration and a bone infarct. Given these findings, a body CT scan was performed which showed multiple mesenteric adenopathies and a large retroperitoneal mass. A lymph node biopsy confirmed a B cell lymphoma. Monoarthritis with no systemic manifestations represents a highly uncommon form of presentation of lymphoma. Moreover it usually affects inferior limbs, particularly in the presence of bone infarction.

Renovascular hypertension leads to DNA damage and apoptosis in bone marrow cells.

Angiotensin II (Ang II), which plays a pivotal role in the pathophysiology of the two-kidney, one-clip (2K1C) Goldblatt hypertension, has been associated with augmented generation of reactive oxygen species (ROS) in some cells and tissues. In the present study, we evaluated the influence of 2K1C hypertension on oxidative stress, DNA fragmentation, and apoptosis of bone marrow (BM) cells. Two weeks after the renal artery clipping or Sham operation, flow cytometry analysis showed a higher production of superoxide anions (approximately sixfold) and hydrogen peroxide (approximately twofold) in 2K1C hypertensive than in Sham normotensive mice. 2K1C mice also showed an augmented DNA fragmentation (54%) and apoptotic cells (21%). Our data show that the 2K1C renovascular hypertension is characterized by an increased production of ROS, DNA damage, and apoptosis of BM, which is a fundamental source of the cells involved in tissue repair.

Telomere dysfunction and tumor suppression responses in dyskeratosis congenita: balancing cancer and tissue renewal impairment.

Dyskeratosis congenita (DC) encompasses a large spectrum of diseases and clinical manifestations generally related to premature aging, including bone marrow failure and cancer predisposition. The major risk factor for DC is to carry germline telomere-related mutations - in telomerase or telomere shelterin genes - which results in premature telomere dysfunction, thus increasing the risk of premature aging impairments. Despite the advances that have been accomplished in DC research, the molecular aspects underlying the phenotypic variability of the disease remain poorly understood. Here different aspects of telomere biology, concerning adult stem cells senescence, tumor suppression and cancer are considered in the context of DC, resulting in two translational models: late onset of DC symptoms in telomere-related mutations carriers is a potential indicator of increased cancer risk and differences in tumor suppression capacities among the genetic subgroups are (at least partial) causes of different clinical manifestations of the disease. The limitations of both models are presented, and further experiments for their validation, as well as clinical implications, are discussed.

Bone marrow edema patterns in the ankle and hindfoot: distinguishing MRI features.

OBJECTIVE: Many disorders produce similar or overlapping patterns of bone marrow edema in the ankle. Bone marrow edema may present in a few hindfoot bones simultaneously or in a single bone. The purpose of this pictorial essay is to provide guidelines based on clinical history and specific MRI patterns and locations to accurately identify the cause of ankle bone marrow edema. We will first focus on bone marrow edema in general disease categories involving multiple bones, such as reactive processes, trauma, neuroarthropathy, and arthritides. A discussion of bone marrow edema in individual bones of the ankle and hindfoot including the tibia, fibula, talus, and calcaneus will follow. Helpful hints for arriving at the correct diagnosis will be provided in each section. CONCLUSION: After review of this article, radiologists should be able to use their knowledge of clinical history and specific MRI patterns and locations to accurately distinguish between the various causes of bone marrow edema in the ankle and hindfoot.

Bone marrow necrosis related to paracoccidioidomycosis: the first eight cases identified at autopsy.

AIMS: To report the first eight bone marrow necrosis (BMN) cases related to paracoccidioidomycosis (PCM) from patient autopsies with well-documented bone marrow (BM) histology and cytology. METHODS AND RESULTS: A retrospective evaluation was performed on BM specimens from eight autopsied patients from Botucatu University Hospital with PCM-related BMN. Relevant BMN literature was searched and analysed. CONCLUSIONS: All eight patients had acute PCM. Six had histological only (biopsies) and two cytological only (smears) specimens. Five biopsy specimens revealed severe and one mild coagulation patterned necrotic areas. Five had osteonecrosis. The cytological specimens also showed typical BMN patterns. Paracoccidioides brasiliensis yeast forms were visible within necrotic areas in all cases.

Infiltrative myelopathy by paracoccidioidomycosis. A review and report of nine cases with emphasis on bone marrow morphology.

AIMS: To report nine additional well-defined cases with infiltrative myelopathy by paracoccidioidomycosis (PCM), to describe the specific lesions and infection-related stromal abnormalities, to review the literature on this type of involvement and to introduce a new cause of granulomatous lesions of bone marrow. METHODS AND RESULTS: Different bone marrow specimens were studied (aspirated smears, aspirated clots, biopsy imprints and biopsies) from nine patients with acute or subacute forms of PCM known to have PCM infiltrative myelopathy. CONCLUSIONS: The biopsy specimens were the best for demonstrating bone marrow involvement by PCM. The lesions varied from compact and focal granulomas with few fungal cells to numerous disseminated fungal cells within a loose granulomatous inflammatory reaction, with a continuum between these extremes suggesting a spectrum of immune response to the fungi. Other findings such as bone marrow fibrosis, parenchymal coagulative necrosis and bone necrosis were also observed in the affected areas.

Impact of shock and fluid resuscitation on the morphology and apoptosis of bone marrow: an experimental study.

BACKGROUND: We hypothesized that bone marrow failure after hemorrhagic shock might be secondary to impaired apoptosis regulation. Our objective was to assess the morphologic alterations and the rate of apoptosis in bone marrow after hemorrhagic shock and resuscitation. METHODS: Under pentobarbital anesthesia, Wistar rats (n = 70) underwent femoral vessel cannulation. The hemorrhagic shock model involved a controlled retrieval of blood, maintaining mean blood pressure at 40 +/- 5 mm Hg during 50 minutes. During the resuscitation period, lactated Ringer's (twice the blood volume retrieved, group LR) or NaCl 7.5% (4 mL/kg, group HS) was infused, followed by the previously retrieved blood. Bone marrow was collected through left femoral puncture. Morphology was assessed by Leishmann-stained smears, and apoptosis was assessed through terminal deoxynucleotide transferase-mediated dUTP nick-end labeling assay. Analysis of variance and Tukey's test were applied for statistical treatment, considering p < 0.05 as significant. RESULTS: LR animals presented a statistically significant decrease in the lymphocytic series (LR, 24.2 +/- 4.2%; Sham, 55.1 +/- 6.6%), together with an increase in the percentage of granulocyte (LR, 51.4% +/- 2.3%; Sham, 31.5 +/- 2.9%) and monocyte precursors (LR, 7.3 +/- 1.3%; Sham, 3.3 +/- 1.1%), detected 72 hours after shock (p < 0.05). Both LR and HS groups presented a significant increase in apoptosis, when compared with the sham group (LR, 13.1 +/- 0.5%; HS, 12.2 +/- 0.7%; Sham, 6.8 +/- 0.4%). The alterations detected in the bone marrow morphology of LR group were not observed in HS animals. CONCLUSION: There was an increase in bone marrow apoptosis after hemorrhagic shock. The type of resuscitation scheme used did influence bone marrow morphology.

Citomorfología de médula ósea en pacientes con SIDA / Bone marrow cytomorphology in patients with AIDS

El objetivo de éste estudio fue analizar las características citomorfológicas de la médula ósea de pacientes con SIDA y correlacionarlas con las alteraciones hematológicas periféricas. Se incluyeron 29 pacientes en quienes el aspirado de médula ósea se realizó con las técnicas habituales y el hemograma se efectuó en el mismo momento. Los motivos de petición del aspirado medular fueron: citopenias (58 por ciento), fiebre de origen desconocido (38 por ciento) e investigación de patología tumoral (4 por ciento). El 92 por ciento de los pacientes presentó citopenias: anemia (52 por ciento), trombocitopenia (4 por ciento), bicitopenia (16 por ciento) y pancitopenia (28 por ciento). Un caso mostró fibrosis medular y no pudo ser evaluado. En los casos restantes, se constató celularidad medular normal (64 por ciento) o aumentada (36 por ciento). La relación mieloide/eritroide (M/E) fue: disminuida (46 por ciento), normal (36 por ciento) o aumentada (18 por ciento). Las principales anomalías citomorfológicas fueron: diseritropoyesis con rasgos megaloblásticos (63 por ciento), disgranulopoyesis con asincronismo madurativo entre núcleo y citoplasma, hipo/hipergranularidad y desviación a la derecha (48 por ciento) y distrombopoyesis con megacariocitos hipolobulados o núcleos desnudos de megacariocitos (41 por ciento). Además, se constató aumento de células plasmáticas (39 por ciento, algunas con morfología anormal), de eosinófilos (14 por ciento), de linfocitos (11 por ciento) y presencia de células tipo Gaucher. Las citopenias estuvieron asociadas a una médula ósea normo o hipercelular y la anemia a una meyor frecuencia de displasia eritroide. En 6/28 pacientes (21 por ciento), la médula ósea proporcionó diagnóstico morfológico. En conclusión, los hallazgos realizados indican que, si bien algunas anomalías citomorfológicas de la médula ósea no son específicas, determinados rasgos morfológicos podrían sugerir infección por VIH (AU)

Citomorfología de médula ósea en pacientes con SIDA / Bone marrow cytomorphology in patients with AIDS

El objetivo de éste estudio fue analizar las características citomorfológicas de la médula ósea de pacientes con SIDA y correlacionarlas con las alteraciones hematológicas periféricas. Se incluyeron 29 pacientes en quienes el aspirado de médula ósea se realizó con las técnicas habituales y el hemograma se efectuó en el mismo momento. Los motivos de petición del aspirado medular fueron: citopenias (58 por ciento), fiebre de origen desconocido (38 por ciento) e investigación de patología tumoral (4 por ciento). El 92 por ciento de los pacientes presentó citopenias: anemia (52 por ciento), trombocitopenia (4 por ciento), bicitopenia (16 por ciento) y pancitopenia (28 por ciento). Un caso mostró fibrosis medular y no pudo ser evaluado. En los casos restantes, se constató celularidad medular normal (64 por ciento) o aumentada (36 por ciento). La relación mieloide/eritroide (M/E) fue: disminuida (46 por ciento), normal (36 por ciento) o aumentada (18 por ciento). Las principales anomalías citomorfológicas fueron: diseritropoyesis con rasgos megaloblásticos (63 por ciento), disgranulopoyesis con asincronismo madurativo entre núcleo y citoplasma, hipo/hipergranularidad y desviación a la derecha (48 por ciento) y distrombopoyesis con megacariocitos hipolobulados o núcleos desnudos de megacariocitos (41 por ciento). Además, se constató aumento de células plasmáticas (39 por ciento, algunas con morfología anormal), de eosinófilos (14 por ciento), de linfocitos (11 por ciento) y presencia de células tipo Gaucher. Las citopenias estuvieron asociadas a una médula ósea normo o hipercelular y la anemia a una meyor frecuencia de displasia eritroide. En 6/28 pacientes (21 por ciento), la médula ósea proporcionó diagnóstico morfológico. En conclusión, los hallazgos realizados indican que, si bien algunas anomalías citomorfológicas de la médula ósea no son específicas, determinados rasgos morfológicos podrían sugerir infección por VIH

Hipoplasia medular associada ao uso de metotrexato, em baixas doses, no lúpus eritematoso sistêmico / Medular hypoplasia related to low doses of methotrexate in systemic lupus erythematosus

A hipoplasia de medula óssea é um efeito colateral incomum do uso de maetotrexato (MTX), em baixas doses, na artrite reumatóide (AR). Fatores de risco para essa complicação são: Hipoalbuminemia, queda do clearance de creatinina, alcoolismo, idade avançada e uso de certas drogas, como a ranitidina ou sulfametoxazol-trimetoprim. Os autores não encontraram relatos desse efeito adverso no lúpus eritematoso sistêmico e apresentam um caso de hipoplasia de medula óssea em paciente com LES em atividade, que estava sendo tratado com baixas doses de MTX. Discutem, também, os fatores de risco para essa complicação

Manifestaciones hematológicas en la infección por VIH / Hematologic manifestations of HIV infection

Las manifestaciones hematológicas en la infección por el virus de la inmunodeficiencia humana son floridas, ya que esta patología ha adquirido particular relevancia en los últimos años por el aumento en la frecuencia de su presentación. Se analizan las distintas formas de compromiso hematológico, así como las indicaciones para la realización de estudios invasivos : biopsia de médula ósea y biopsia ganglionar

Manifestaciones hematológicas en la infección por VIH / Hematologic manifestations of HIV infection

Las manifestaciones hematológicas en la infección por el virus de la inmunodeficiencia humana son floridas, ya que esta patología ha adquirido particular relevancia en los últimos años por el aumento en la frecuencia de su presentación. Se analizan las distintas formas de compromiso hematológico, así como las indicaciones para la realización de estudios invasivos : biopsia de médula ósea y biopsia ganglionar(AU)

Clinical factors predictive of bone marrow involvement in Hodgkin's disease.

The role of bone marrow biopsy in the staging of Hodgkin's disease is undergoing reevaluation. We have studied the relationship of clinical factors to the presence of bone marrow involvement in 130 previously untreated patients with Hodgkin's disease. The presence of fever, spleen enlargement, anemia, leukopenia, poor performance status and poor histologic subgroups were positively correlated with the presence of bone marrow involvement in the univariate analysis. In the multivariate analysis, only fever, spleen involvement, leukopenia and poor histologic subgroups were significant. The predictive value of the absence of fever in regard to the absence of bone marrow involvement was 98%. The likelihood of bone marrow involvement in the absence of all four significant factors was only 0.05%. Patients without these clinical factors should probably not be submitted to a bone marrow biopsy as part of the staging procedures performed in Hodgkin's disease.

Lysinuric protein intolerance characterized by bone marrow abnormalities and severe clinical course.

STUDY OBJECTIVE: To evaluate phenotypic variability of lysinuric protein intolerance in a cohort of nine Italian patients. DESIGN: Retrospective analysis of patient records. SUBJECTS: Nine Italian patients (seven independent families), all originating from southern Italy, observed during the last 14 years. RESULTS: Some of the patients had unique clinical features, including bone marrow abnormalities featuring erythroblastophagocytosis (five patients) and clinical course and the outcome of the disease, have also been observed: respiratory involvement was present in five cases, with a lethal picture of "alveolar proteinosis" in one. Severe kidney involvement, with both glomerular and tubular damage and rapidly progressing to chronic renal failure, has been observed in one case. CONCLUSION: Lysinuric protein intolerance may cause severe multisystem involvement, which requires early and careful monitoring. Some peculiar clinical findings observed in Italian patients point to a genetic heterogeneity of lysinuric protein intolerance.

[Bone marrow granulomas]. / Granulomas en medula ósea.

PURPOSE: To evaluate bone marrow granulomatous lesions in order to establish their etiology. MATERIAL AND METHODS: 2,250 bone marrow biopsies were studied during the period of March 1983-March 1991. Granulomas and/or granulomatous lesions were found in 24 of them (1.06%). A correlation between histological characteristics, special stains: PAS, Ziehl Neelsen and Grocott and cultures were done. Immunohistochemistry was done to evaluate B or T cell-lineage in 4 patients. RESULTS: The 24 patients were biopsied because of the clinical diagnosis of haematological and non-haematological neoplasias, infections, AIDS, sarcoidosis and fever of unknown origin. Bone marrow cellularity ranged from 20% to 75% (M: 49.8%). Myeloid cells were increased in 54% of the cases. The number of granulomas ranged from 1 to 19 (M: 3.9). The epithelioid cells were the predominant component in 66% of the cases. Of the 7 patients with non-Hodgkin's lymphoma, 4 had lymphomatous involvement with granulomatous pattern. These cases showed predominance of lymphoid cells and vessels in addition to epithelioid cells. CONCLUSION: We consider that in order to establish a relationship between infection and granuloma, the identification of a microorganism through a culture is a more reliable test. We couldn't find any morphological characteristic which allowed an etiologic diagnosis of bone marrow granulomas. In case of lesions with a great lymphocytic and vascular proliferation plus the presence of epithelioid cells and fibrosis, NHL with bone marrow involvement with a granulomatous pattern should be strongly considered.