Background: Pancytopenia is a common hematological parameter detected in Hematology department It is important to find out the etiology of pancytopenia for the better management of patient Pancytopenia represents a wide range of age groups and most pancytopenia is purely of bone marrow dysfunction origin However, other causes such as hypersplenism and SLE are also frequently encountered Objectives: To differentiate various causes of pancytopenia, to evaluate the hematological parameters in pancytopenic cases, and to analyze clinical manifestations in cases of pancytopenia Materials and Methods: There were 25 admitted cases of pancytopenia at Affiliated Hospital of Dali University between the calendar year 2013 and 2014 A detail study was done on the basis of age, gender, clinical features, typical laboratory investigations, bone marrow examination, and final diagnosis Results and Conclusions: Among 25 cases studied, megaloblastic anemia was the common cause of pancytopenia and incidence of megaloblastic anemia was higher in males after 5th decade of life Pancytopenic cases age was ranging from 15 to 80 years Fatigue and dizziness were the most common clinical features presented

/pdf/detection-of-pancytopenia-associated-with-clinical-3lpo6oy0jf.pdf

Detection of Pancytopenia Associated with Clinical Manifestation and Their Final Diagnosis

Hemophagocytic lymphohistocytosis (HLH) is commonly associated with infectious diseases or T/NK cell-lymphoma; however that with Hodgkin lymphoma (HL) was rarely reported. Herein, we describe a young male diagnosed with lymphocyte-depleted HL (LD-HL) complicated by HLH as an initial manifestation. He was given high-dose steroid therapy plus recombinant thrombomodulin, and subsequent ABVd (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment. In spite of the achievement of a partial remission treated with one cycle of ABVd, he relapsed after 3 cycles. To our knowledge, the present case is very rare, and more intensive treatment might be needed for the long-term control of HLH-complicated HL.

Lymphocyte-depleted Hodgkin lymphoma complicating hemophagocytic lymphohistiocytosis as an initial manifestation: a case report and review of the literature.

Background: Pancytopenia is the feature of many life threatening conditions. It is described as the deficiency of all three cellular elements of blood resulting in anemia, leucopenia and thrombocytopenia. It is a common hematological entity that we come across in routine practice. A comprehensive clinical, hematological and bone marrow study of patients usually helps in identification of the underlying cause. However in view of a wide array of etiological factors pancytopenia continues to be a challenge. Aspiration of the marrow is an indispensible adjunct to the study of hematopoietic disorders, if performed correctly. It is simple, safe and can be repeated. In case of trephine biopsy the greater value is that it can provide information about the structure of relatively large pieces of marrow. At the same time morphological features of individual cells may be identified by making imprint from the material obtained. Hence the present study was undertaken to evaluate the accuracy of the three components of marrow examination in the diagnosis of hematological disorders presenting with pancytopenia.  Methods: A total of 58 patients presenting with pancytopenia on initial workup requiring bone marrow aspirate and biopsy at MS Ramaiah medical college and hospital, Bangalore were studied from October 2009 to September 2011. Smears were made from the bone marrow aspirate (BMA), stained by Leishmann stain and examined. Touch imprints of bone marrow biopsy (BMB) were made and examined before the tissue was put in fixative (10% neutral buffered formalin) and subsequently processed, embedded in paraffin and sections were prepared and examined. Results: Fifty eight patients with hematological diagnosis of pancytopenia were studied during the period October 2009 to September 2011. The sex distribution of pancytopenia showed male preponderance (60%). Most of the patients were in the age group of 21 - 30 years (25%) and least occurrence was seen in patients more than seventy years (3.45%). The commonest cause of pancytopenia was megaloblastic anemia (39.65%), followed by nutritional anemia (24.13%) and Aplastic anemia (12.06%). Eleven patients (18.9%) were diagnosed on bone marrow biopsy. Forty four out of the total fifty eight cases showed a positive correlation.  Conclusion: The commonest cause of pancytopenia was megaloblastic anemia. Clinical history and bone marrow examination is critical in arriving at the diagnosis. There was a positive correlation of 75.8% between BMA and BMB. Both the procedures were complimentary to each other and should be performed simultaneously along with imprint smears for complete bone marrow workup and evaluation.  doi: http://dx.doi.org/10.4021/jh76w

/pdf/correlation-of-bone-marrow-aspirate-biopsies-and-touch-1d1munxpdp.pdf

Correlation of Bone Marrow Aspirate, Biopsies and Touch Imprint Findings in Pancytopenia

The patient was a 15-year-old girl with severe acute hepatitis. A liver biopsy showed the typical findings of autoimmune hepatitis (AIH). Subsequently, two lineages of cytopenia were found in the patient's peripheral blood. Hemophagocytosis by macrophages was observed in the bone marrow. Virus-, drug- and lymphoma-associated hemophagocytic syndrome (HPS) was ruled out. Therefore, the patient was diagnosed with autoimmune-associated HPS (AAHS). Following the administration of combination therapy with prednisolone and cyclosporine A, both the AAHS and AIH improved. This is the first report of AAHS originating from AIH. The patient was followed up for five years after treatment, and no disease recurrence was detected.

Autoimmune-associated hemophagocytic syndrome originating from autoimmune hepatitis with a successful response to therapy.

Although hemophagocytic syndrome is a rare clinical condition, it is associated with high mortality and the number of cases described in the literature has progressively increased. The diagnosis of hemophagocytic syndrome is made on the basis of a finding of hemophagocytosis. Sarcoidosis is a highly prevalent disease whose course and prognosis might correlate with the initial clinical presentation and the extent of the disease. We report the case of a patient with long-standing sarcoidosis who presented with intermittent fever and fatigue. The diagnosis of hemophagocytic syndrome was made by bone marrow aspiration, and specific treatment was ineffective. This is the third case of sarcoidosis-related hemophagocytic syndrome reported in the literature and the first reported in Latin America. All three cases had unfavorable outcomes.

/pdf/sindrome-hemofagocitica-devido-a-sarcoidose-pulmonar-4s8mz7quyg.pdf

Síndrome hemofagocítica devido a sarcoidose pulmonar

Background. Hemophagocytic syndrome (HPS) is characterized by a hyperinflammatory reaction followed by alteration in cytotoxic function of Th1 lymphocytes and natural killer cells. We report a rare case of a patient that presented with fever and pancytopenia due to HPS associated with Hodgkin's lymphoma (HL). Case Report. A 69-year-old Caucasian woman was admitted presenting with complaints of fever, seizures, and low back pain that had lasted for two weeks. Laboratorial data showed pancytopenia. Bone marrow biopsy revealed infiltration by Reed-Sternberg cells and hemophagocytosis signs. Imaging studies showed mediastinal lymph nodes (stage IV B). She had been treated with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) followed by a good response. Conclusion. HPS associated with HL is a very rare and lethal disease, with mortality rates of about 15% to 60%. The prompt diagnosis of the underlying lymphoma may be an important strategy for optimizing the clinical approach and outcome.

/pdf/hemophagocytic-syndrome-associated-with-hodgkin-s-lymphoma-ae8grahyto.pdf

Hemophagocytic Syndrome Associated with Hodgkin's Lymphoma First Presenting as Fever and Pancytopenia.

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Hemophagocytic Syndrome Associated with Hodgkin's Lymphoma First Presenting as Fever and Pancytopenia.