Hemophagocytic lymphohistiocytosis as a presentation of inflammatory bowel disease / Linfohistiocitosis hemofagocítica como presentación de la enfermedad inflamatoria intestinal

Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is considered a medical emergency that should be recognized in patients with fever, splenomegaly, and progressive deterioration of the general condition. Laboratory findings include cytopenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. For HLH diagnosis, it is essential, although not mandatory, to perform a bone marrow biopsy. Given its nature, secondary causes of HLH should be sought, mainly infections, hemato-oncological disorders, autoimmune diseases, and auto-inflammatory conditions. Case report: We present the case of a female adolescent who presented with fever and lower gastrointestinal bleeding. Upon admission, acute liver failure and pancytopenia were documented. A bone marrow aspirate was performed, which revealed hemophagocytosis; other tests confirmed HLH diagnosis. During the diagnostic approach, inflammatory bowel disease was diagnosed. The patient received first-line treatment with an adequate response. Conclusions: Inflammatory bowel disease can be considered a cause of secondary HLH, particularly in patients with suggestive symptoms, such as digestive bleeding in the absence of other secondary causes of HLH.

Resumen Introducción: La linfohistiocitosis hemofagocítica (LHH) es considerada una urgencia médica que debe reconocerse en pacientes con deterioro progresivo del estado general, fiebre, pancitopenia y esplenomegalia. Los hallazgos de laboratorio incluyen citopenia, hipertrigliceridemia, hipofibrinogenemia e hiperferritinemia. Para su diagnóstico es importante, aunque no obligatoria, la realización de aspirado de médula ósea. Dada su naturaleza, se deben buscar causas secundarias de LHH, principalmente enfermedades infecciosas, hematooncológicas, autoinmunitarias y autoinflamatorias. Caso clínico: Se presenta el caso de una adolescente que inició con fiebre y sangrado digestivo bajo. A su ingreso, se documentó falla hepática aguda y pancitopenia. Se realizó aspirado de médula ósea y se encontró hemofagocitocis; el resto de los exámenes concluyeron LHH. Durante su abordaje se diagnosticó enfermedad inflamatoria intestinal. La paciente recibió tratamiento de primera línea con adecuada respuesta. Conclusiones: La enfermedad inflamatoria intestinal puede considerarse como una causa secundaria de LHH, en particular en pacientes con clínica sugestiva, como es el sangrado digestivo, en ausencia de otras causas secundarias de LHH.

Necrobiotic Xanthogranuloma Coexists with Diffuse Normolipidemic Plane Xanthoma and Multiple Myeloma

Pratique du myélogramme en 10 ans au laboratoire d'Hématologie du Centre Hospitalier Universitaire Joseph Ravoahangy Andrianavalona (CHUJRA) Antananarivo, Madagascar

Une étude rétrospective descriptive a été menée sur 2338 myélogrammes effectués au laboratoire d'hé-matologie du centre hospitalier universitaire Joseph Ravoahangy Andrianavalona Antananarivo de janvier 2008 à décembre 2017. L'objectif principal était de décrire les résultats des myélogrammes pendant cette période.L'étude a montré que la fréquence moyenne de demande de myélogramme est de 234 par an. L'âge moyen des patients était de 33,30 ans avec des extrêmes de 29 jours à 82 ans. Une prédominance masculine a été notée avec un sex ratio H/F de 1,2.Les anomalies de l'hémogramme ont constitué le motif principal de prescription des myélogrammes (58,08%). La pancytopénie était la plus fréquente.Les diagnostics révélés par les analyses des myélogrammes étaient le plus fréquemment des hémopathies malignes (58,1%), dont 32,97% de leucémies aigues.Parmi les myélogrammes analysés, 10% étaient nor-maux. La confrontation clinico-biologique est de mise pour une bonne pratique du myélogramme

Clinical Effects of Hypomethylating Agents in Patients with Newly Diagnosed Myelodysplastic Syndrome Who Received DNA-Damaging Chemotherapy for Metastatic Breast Cancer / 한국유방암학회지

Promyelocytic Leukemoid Reaction: Unusual Findings in a Patient with Sepsis

Neutrophilic leukemoid reaction may occur in many situations, including hemolysis, malignancy, infection, and exposure to certain toxins. It usually shows morphological overlap with chronic myeloid leukemia in which promyelocytes are not majorly associated. Here, we present a case of promyelocytic leukemoid reaction in a patient with sepsis. A 28-year-old man was admitted for renal stone removal. After percutaneous nephrolithotomy, his condition deteriorated with fever (37.8℃), tachycardia (130/min), acute renal failure, pleural effusion, and pulmonary edema. Complete blood count indicated a white blood cell count of 73.39×10⁹/L including 82% promyelocytes, hemoglobin 8.9 g/dL, and platelet count of 85×10⁹/L. A bone marrow aspirate showed that promyelocytes accounted for 73.8% of all nucleated cells. Following bone marrow examination, treatment with all-trans retinoic acid (ATRA) was started immediately. Reverse transcription polymerase chain reaction (RT-PCR) study revealed the absence of PML-RARA (promyelocytic leukemia-retinoic acid receptor alpha) and other RARA (retinoic acid receptor alpha) rearrangements. Once the chromosome analysis of bone marrow cells demonstrated the normal karyotype, ATRA was discontinued.

Discomfort and Bleeding in Relation to Bedrest Time after Bone Marrow Examination among Hemato-oncology Patients / 임상간호연구

PURPOSE: The purpose of this study was to investigate hemato-oncology patients' discomfort and bleeding in relation to the bedrest time after bone marrow examination. METHODS: A descriptive correlational study was conducted. The data were collected using self-report questionnaire from total of 131 patients who underwent bone marrow examination from January 2017 to September 2017. Data were analyzed with descriptive statistics, Wilcoxon Signed-rank test, McNemar's test and logistic regression. RESULTS: The level of discomfort after 4 hours of bedrest was significantly higher when compared to 2 hours of bedrest(p<.001). The occurrence of bleeding after 2 hours of bedrest was significantly higher than 4 hours of bedrest(p<.001), however the degree of bleeding was slight. No bleeding occurred in 84% of the patients after 2 hours of bedrest. CONCLUSION: The results of this study demonstrated that shortening the bed rest time after bone marrow examination was helpful in improving the patient's well-being. Bedrest time could be shortened according to the site of bone marrow examination and patient's condition.

Significance of Bone Marrow Unclassifiable Cells in Diagnosis of Fever of Unknown Origin / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical significance of bone marrow unclassifiable cells in diagnosis of fever of unknown origin(FUO).@*METHODS@#The clinical data of 60 patients with FUO admitted in the first affiliated hospital of Xi'an Jiaotong university from June 2014 to May 2016 were collected, and 60 patients with FUO were divided into 2 group: group A(30 cases) in which the unclassifiable cells in bone marrow were observed by bone marrow examination, and group B(30 cases) in which the unclassifiable cells in bone marrow not were found by bone marrow examination. The clinical characteristics, bone marrow features, immunophenotypes of bone marrow cells and prognosis of patients in 2 groups were analyzed retrospectively.@*RESULTS@#Out of 30 patients in group A, 18 were diagnosed as malignant tumors including 12 cases of lymphoma, while out of 30 patients in group B, 5 cases were diagnosed as malignant tumor, including 3 cases of lymphoma. For the patients with non-tumor diseases, the bone marrow unclassifiable cells disappeared after the patients condition was improved.@*CONCLUSION@#The bone marrow examination including the smear and biopsy shonld be performed routinely for the patients with FUO. If the unclassifiable cells are observed morphologically in bone marrow of patients with FUO, the disease of patients should be considered as malignant tumor, especially, lymphoma.

Leukemic Pleural Effusion in Acute Promyelocytic Leukemia: A Case Report

In patients with acute myeloid leukemia (AML), pleural effusion may be attributed to various factors, including infection, hypoalbuminemia, and renal failure. However, leukemic infiltration of the pleural fluid is rarely reported and poorly understood. Extramedullary diseases have been reported with increasing frequency as the survival rates of patients with AML have increased. However, the reported prognostic effects of leukemic pleural effusion in patients with AML range from none to a worse prognosis. Here, we report a case of acute promyelocytic leukemia (APL) in a patient exhibiting leukemic pleural effusion with fluorescence in situ hybridization (FISH) results indicating the presence of the PML-RARA fusion gene. A 52-year-old man presented with pancytopenia, dyspnea, and fever. He had a medical history of hypertension, end-stage renal disease, and hepatitis B virus-related liver cirrhosis. A peripheral blood smear revealed the presence of multiple abnormally hypergranular promyelocytes. White blood cell differential counts were not performed due to severe pancytopenia. A bone marrow examination, immunophenotyping analysis, and cytogenetic and molecular studies revealed APL. The patient was treated with all-trans retinoic acid immediately after abnormal promyelocytes were observed in the peripheral blood smear, but induction chemotherapy was delayed because of his poor condition. His persistent dyspnea and abdominal discomfort led to a thoracentesis and the observation of abnormal promyelocytes that were positive for PML-RARA fusion gene by FISH. To our knowledge, this is the first report of leukemic pleural infiltration with PML-RARA fusion gene-positivity via FISH.

Bone Marrow Morphology of Multiple Myeloma with Non-bone-related Extramedullary Disease / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To explore the morphological characteristics of bone marrow cells of multiple myeloma with non-bone-related extramedullary disease and thier clinical significance.</p><p><b>METHODS</b>Bone marrow smears, peripheral blood smears and bone marrow biopsy sections as well as thier examination results of 20 cases of multiple myloma with non-bone-related extramedullary disease were collected at initial diagnosis in First Affiliated Hospital of Xi'an Jiaotong University from March 2013 to March 2016, and morphological characterisistes of bone marrow cells were analysed in combination with clinical data.</p><p><b>RESULTS</b>The morphology of plasma cells in 20 patients showed 2 cytologic subtypes: primitive cell type (16 cases) and pleomorphic type (4 cases). Immature plasma cells were found in the 5 patients' peripheral blood smear, accounting for about 1%-4% of the number of peripheral blood cells. In bone marrow tissue, plasma cells hyperproliferated with nodular and packed type, and secondary myelofibrosis counted for 12 cases (60%). 13 MM patients whose non-bone-related extramedullary disease occurred during therapy were divided into 2 groups according to the marrow fibrosis density. The median time from diagnosis of MM to extramedullary lesions resulting from fibrosis 0-1 grade and 2-3 grade was 23.7±3.7 months and 10.5±3.2 months ahead of the former(P=0.025).</p><p><b>CONCLUSION</b>Bone marrow plasma cell morphology of multiple myeloma with non-bone-related extramedullary disease at the initial diagnosis is mostly immature type, and plasma cells proliferate with nodular and packed type and accompanied by different degree of fibrosis. The degree of myelofibrosis indirectly reflects the degree of proliferation and malignancy of the bone marrow plasma cells, which maybe possess some value in predicting extramedullary disease in the early stages of the MM.</p>

Prevalence and Implications of Bone Marrow Involvement in Patients with Gastric Mucosa-Associated Lymphoid Tissue Lymphoma

BACKGROUND/AIMS: Mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach is an uncommon disease. Bone marrow involvement is reported even in patients with only a mucosal lesion. We evaluated the prevalence and risk factors of marrow involvement and its implications for diagnosis and treatment. METHODS: In total, 132 patients who were diagnosed with gastric MALT lymphoma at the National Cancer Center in Korea between January 2001 and December 2016 were enrolled in the study. The patient data were collected and analyzed retrospectively. RESULTS: Of the 132 patients, 47 (35.6%) were male, with a median age of 52 years (range, 17 to 81 years). The median follow-up duration was 48.8 months (range, 0.5 to 169.9 months). Helicobacter pylori infection was detected in 82 patients (62.1%). Most patients (80.3%) had stage IE1 according to the modified Ann Arbor staging system. Ninety-two patients underwent bone marrow evaluation, and four patients (4.3%) had marrow involvement. Of these patients, one presented with abdominal lymph node involvement, while the other three had stage IE1 disease if marrow involvement was disregarded. All three patients had no significant symptoms and were monitored after local treatment without evidence of disease aggravation. CONCLUSIONS: Bone marrow involvement was found in 4.3% of the patients with gastric MALT lymphoma. Bone marrow examination may be deferred because marrow involvement does not change the treatment options or outcome in gastric MALT lymphoma confined to the stomach wall.

Aplastic Anemia and Severe Myelosuppression with Boceprevir or Simeprevir-Containing Hepatitis C Virus Treatment

ABSTRACT The addition of the new protease inhibitors (PIs) to peg-interferon (IFN) and ribavirin (RBV), approved for chronic hepatitis C, has clearly improved sustained virological response (SVR) rates although several adverse events have been reported with this regimens, including mild hematological toxicity. Moreover, severe pancytopenia and aplastic anemia during triple therapy with telaprevir has recently been described in seven patients. We report here two cases of severe agranulocytosis/aplastic anemia using boceprevir or simeprevir in interferon-based combination and 2 additional cases of severe myelosupression in IFN-free therapy with sofosbuvir and simeprevir plus RBV. Our observations suggest that PIs could have a sort of class-effect in developing severe hematologic toxicity or, at least, an additive interaction with other potentially myelotoxic agents such as IFN or RBV that are used in the classical regimens against HCV. Unfortunately, the mechanisms behind this phenomenon are currently unknown. In conclusion, given the lifethreatening character of these complications, close monitoring is mandatory in patients under PIs based therapy to promptly detect serious hematological toxicities and to carefully evaluate treatment discontinuation. Prospective studies assessing the usefulness of RBV in the era of new IFN-free combinations are needed.

Metastatic breast cancer with osteolytic skull lesions suspected to be multiple myeloma

We report a case of breast cancer with osteolytic skull lesions which mimicked osteolytic lesions in multiple myeloma. A 60-year-old female was admitted to our hospital due to confused mentality. Laboratory tests showed the findings of an increased calcium level, kidney failure, and anemia. Multiple osteolytic lesions were detected in the ribs, spine, humerus, and pelvis on X-rays. The skull showed the punched out sign. Accordingly we initially suspected multiple myeloma; however, monoclonal protein was not detected in serum and urine and the number of plasma cells was not increased in bone marrow examination. In bone marrow examination, metastatic cancer was detected and biopsy revealed breast cancer. Finally, breast cancer with multiple metastases including those to bone, liver, and lung was diagnosed. Therefore, when a patient presents with multiple osteolytic lesions, we need to consider metastasis from solid cancer in the differential diagnosis as well as multiple myeloma.

A Sporadic Case of Epstein Syndrome: A Rare Cause of Refractory Thrombocytopenia / 대한내과학회지

A 37-year-old female presented to our hospital with a history of bleeding episodes (excessive bleeding after tooth extraction, gum bleeding, easy bruising, and excessive menstruation) and severe thrombocytopenia (2,000/µL). She had no family history of bleeding tendency or thrombocytopenia. No peripheral lymphadenopathy or splenomegaly was noted. The patient's white blood cell count was normal; hemoglobin was 9.7 g/dL. A peripheral blood smear showed markedly decreased platelets, with occasional giant or large platelets. Bone marrow examination found increased megakaryocytes. The patient also complained of hearing difficulty; a hearing test indicated sensory-neural hearing impairment. Her thrombocytopenia was refractory to treatment with glucocorticosteroids, intravenous gamma-globulin, and danazol. In the 13 years following her initial presentation, the patient required anti-hypertensive treatment, a hearing-aid for progressive hearing loss, and started maintenance kidney dialysis. Her clinical history of refractory thrombocytopenia, progressive hearing impairment, and renal failure suggested myosin heavy chain 9 gene-related congenital syndrome (Epstein syndrome), which was confirmed by the presence of a heterozygous deletion mutation, c.221_223del, (p.Lys74del) in peripheral leukocyte deoxyribonucleic acid.

Mieloma múltiple en paciente joven / Multiple Myeloma in Young Patient

Introducción: el mieloma múltiple es una enfermedad hematológica maligna caracterizada por una proliferación de células plasmáticas en la médula ósea. La edad más común del inicio es entre 65 y 70 años, sin embargo se documentan casos en jóvenes en formas cada vez más graves. Presentación de caso: hombre de 31 años, que cursa con dolores óseos generalizados, pérdida de peso y adinamia, de 6 meses de evolución. Se constatan cifras de creatinina y calcio sérico elevadas durante su hospitalización, presenta proteína de Bence-Jones positiva para cadenas ligeras Kappa, y se realiza biopsia de médula que concluye en mieloma plasmoblástico. Conclusiones: se comienza tratamiento con citostático asociado a esteroides. La presentación antes de los 40 años es infrecuente y el pronóstico, sombrío, a pesar del tratamiento oncoespecífico(AU)

Introduction: Multiple myeloma is a malignant hematologic disease characterized by a proliferation of plasma cells in the bone marrow. The most common age of onset is between 65 and 70 years, however cases are documented in young people in increasingly severe forms. Case presentation: A case of a 31-year-old man, with generalized bone pain, weight loss and adynamia, of 6 months of evolution is presented here. Serum creatinine and serum calcium levels are high during hospitalization, Bence-Jones protein is positive for Kappa light chains, and marrow biopsy is performed, which concludes in plasmoblastic myeloma. Conclusions: Treatment with cytostatic associated with steroids is started. This onset before age 40 is not frequent, and prognosis is bleak, despite the specific oncology treatment(AU)

Avaliação da confiabilidade entre dois observadores em exames citopatológico e imunocitoquímico de aspirado de medula óssea no diagnóstico da leishmaniose visceral canina / Reliability evaluation between two evaluators in the cytopathologic and immunocytochemical exams from bone marrow aspirate in the canine visceral leishmaniasis diagnosis

Visceral leishmaniasis is a zoonosis in which the dog appears as the main source of infection in urban areas. Its diagnosis is complex and the cytopathological exam is a fast and cheap alternative to parasite direct visualization and its sensitivity can be increased by immunocytochemistry, though with a higher cost. The accuracy of such methods is dependent on the microscopist's experience and therefore, this study evaluated the reliability of such techniques between two observers, from bone marrow aspirates of 50 dogs from an endemic area for the disease. The parasitological culture in Novy-MacNeal-Nicolle medium was used as the reference standard. Among the main findings, the sensitivities obtained by observers I and II were respectively 62.5% and 37.5%, while specificities were 81.1% and 100%. On immunocytochemistry evaluation, the sensitivity was 0% for both evaluators and the specificity 97.3% and 100%. The agreement between evaluators was weak (κ = 0.167) for the cytopathological test and it could not be evaluated for immunocytochemistry, for which there was no detection by the evaluator II. The agreements among the diagnostic methods and the standard reference for the observer I were reasonable (κ = 0.364) for cytopathological examination and bad (κ = -0.041) for immunocytochemistry. For observer II, such agreement could be assessed only for the cytopathological test, being moderate (κ = 0.497). The results point to the possible expertise difference between evaluators, with the evaluator II demonstrating greater experience when interpreting the citopathological test. Although there was the expected sensitivity increase with immunocytochemistry, the technique used in this study was not effective for the diagnosis of infection, regardless of the evaluator.(AU)

Diagnostic Value of Combined Examination Using 5 Technigues for Myelodysplastic Syndrome / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the potential value of combined examinations of peripheral blood smear, bone marrow smear, bone marrow biopsy, chromosome banding analysis and flow cytometry (FCM) in the diagnoisis of myelodysplastic syndromes.</p><p><b>METHODS</b>A total of 105 MDS patients who were admitted in our hospital from May 2013 to May 2015 and were diagnosed as MDS according to the criteria formulated by WHO were enrolled in this study. The accordance rate of diagnosis by the double test (peripheral blood smear plus bone marrow smear), triple test (above mentioned 2 tests plus bone marrow biopsy), quadruple test (above 3 tests plus chromosome banding analysis), and quintuple test (above 4 plus FCM) was amalyzed and compared.</p><p><b>RESULTS</b>Among the 105 MDS patients, the diagnosis accordance rate was 70.48% for double tests, while 83.81%, 84.76% and 93.33% for triple, quadruple and quintuplet tests, respectively which were significantly higher than that for double tests (peripheral blood smear plus bone marrow smear) (P < 0.05).</p><p><b>CONCLUSION</b>The combined examination of the 5 methods can improve the accuracy of MDS diagnosis.</p>

Comparison of Curative Effects of HAG and CAG Regimens for Patients with AML and Medium/High-Risk MDS / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To study the curative effect of HAG and CAG regimens for patients with acute myelocytic leukemia (AML) and high/medium-risk myelodysplastic syndrome (MDS).</p><p><b>METHODS</b>Fifty two patients from January 2010 to January 2014 were enrolled in this study, 32 were diagnosed with AML and 20 with MDS. All the patients were divided into 2 groups: 26 in HAG group (26 cases) and another 26 in CAG group (26 cases). The bone marrow examination, remission rate, PFS, OS and side reaction rates were compared between 2 groups.</p><p><b>RESULTS</b>After treatment, the bone marrow hyperplasia and juvenile cells were decreased significantly. In HAG group, the remission rate was 57.69% and that was 76.92% in CAG group, the difference between these 2 groups was statistically significant (P<0.05), but the survival time was not statistically significant different between 2 groups (P>0.05). The incidence of side reaction in HAG group was 11.54%, that in CAG group was 7.69%, there was no statistically significant difference (P>0.05).</p><p><b>CONCLUSION</b>Both CAG and HAG regimens have shown significant curative effects for acute myelocytic leukemia and high/medium-risk myelodysplastic syndrome.</p>

Application of Cytogenetic Test for Diagnosis of Bone Marrow Involvement in Patients with Non-Hodgkin's Lymphoma / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the role of cytogenetic analysis in the detection of bone marrow (BM) involvement in patients with non-Hodgkin's lymphoma (NHL).</p><p><b>METHODS</b>The bone marrow samples of 74 patients with NHL were detection by using morphology, cytogenetic test, flow cytometry and molecular biological assay. The detected results of morphology, cytogenetic test, flow cytometry and molecular biological assay alone and thier combined detection were compared, the detective rate and consistencies of the 4 methods were analyzed.</p><p><b>RESULTS</b>The detection rates of BM involvement by using morphology, cytogenetic, flow cytometry, and molecular biological assays were 21.6%, 17.6%, 23.0% and 33.8% respectively. The detective rate was enhanced to 44.6% by combining the 4 methods. Cytogenetic test showed the result consistent with the other methods.</p><p><b>CONCLUSION</b>Although cytogenetic test shows a lower detective rate than the other methods, but in some patients the cytogenetic test can detect the abnormality of bone marrow which can not be detected by other methods alone, the combination test of 4 detection methods can enhance the detectable rate of BM involvement.</p>

Acute Lymphoblastic Leukemia Diagnosed in a Patient with Acromegaly / 대한내과학회지

Acromegaly is a rare disorder caused by excessive amounts of growth hormone. The incidence of colorectal, breast, and thyroid carcinomas is increased in acromegaly. However, there have been few reports on hematological malignancies in acromegaly. We describe a patient who developed acute lymphoblastic leukemia during the course of acromegaly. A 35-year-old woman presented in February 2012 with unexplained lactation and amenorrhea for 4 months. Her growth hormone level was 12.6 microg/L, insulin-like growth factor 1 592.26 ng/mL, and prolactin 242 microg/L. A pituitary macroadenoma secreting GH and prolactin causing acromegaly was diagnosed. Considering her fertility, the dopamine agonist cabergoline 0.5 mg was administered in March 2012. In February 2014, she presented with cytopenia (hemoglobin 12.2 g/dL, white cell count 2.69 x 10(9)/L, platelets 39 x 10(9)/L) and hepatosplenomegaly. A bone marrow examination showed acute B cell lymphoblastic leukemia. She underwent chemotherapy and bone marrow transplantation. A follow-up bone marrow biopsy showed remission.

Mixed-phenotype acute leukemia treated with decitabine

No abstract available.