Macrophage elimination in bone marrow by dexamethasone palmitate is associated with successful engraftment in patients with hemophagocytic syndrome

Typhoid fever and paratyphoid fever (also known as enteric fever) are severe systemic illnesses caused by salmonella typhi and S. paratyphi respectively. Enteric fever is prevalent in developing countries including Nepal, where it still remains as a major health problem. There have been reports of pancytopenia with enteric fever which has been attributed to mechanisms like bone marrow suppression, infection associated hemophagocytic syndrome and disseminated intravascular coagulation. We report here a case of severe pancytopenia in enteric fever as a result of bone marrow suppression due to systemic infection. Keywords: enteric fever, pancytopenia, bone marrow suppression

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Secondary Hemophagocytic Syndrome: The Importance of Clinical Suspicion

Case Reports in Hematology ◽

10.1155/2014/958425 ◽

2014 ◽

Vol 2014 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Cristina Oliveira ◽

Sérgio Chacim ◽

Isabel Ferreira ◽

Nelson Domingues ◽

José Mário Mariz

Keyword(s):

Bone Marrow ◽

Natural History ◽

Autoimmune Diseases ◽

Diagnostic Criteria ◽

Immune Activation ◽

Hemophagocytic Syndrome ◽

Clinical Suspicion ◽

Genetic Mutations ◽

Familial Disorder ◽

Spleen Enlargement

Hemophagocytic syndrome is a rare and potentially fatal disorder characterized by pathological immune activation associated with a primary familial disorder, genetic mutations, or occurring as a sporadic condition. The latter can be secondary to infections, malignancies, or autoimmune diseases. Clinically, patients present signs of severe inflammation, with unremitting fever, cytopenias, spleen enlargement, phagocytosis of bone marrow elements, hypertriglyceridemia, and hypofibrinogenemia. Increased suspicion is determinant to timely initiate treatment in an attempt to alter the natural history. The authors present three clinical cases of this syndrome, with a brief review of the diagnostic criteria and treatment.

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Hemophagocytic Syndrome in a Case of Splenic Large B-Cell Lymphoma

Tumori Journal ◽

10.1177/030089169608200623 ◽

1996 ◽

Vol 82 (6) ◽

pp. 621-624 ◽

Cited By ~ 2

Author(s):

Gualtiero Büchi ◽

Giuseppe Termine ◽

Renzo Orlassino ◽

Mauro Pagliarino ◽

Roberto Boero ◽

...

Keyword(s):

Bone Marrow ◽

Lymph Nodes ◽

B Cell ◽

Diagnostic Criteria ◽

Hemophagocytic Syndrome ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Large B Cell Lymphoma ◽

Peripheral Lymph ◽

Large B Cell

A case of splenic large B-cell lymphoma with hemophagocytic syndrome is reported. The difficulties of diagnosis are emphasized especially when peripheral lymph nodes or bone marrow lymphomatous infiltration are not present. Diagnostic criteria for hemophagocytic syndrome and their relationship with the pathogenesis of the disease are also stressed.

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Hemophagocytic Lymphohistiocytosis and Gastrointestinal Bleeding: What a Surgeon Should Know

Case Reports in Surgery ◽

10.1155/2015/745848 ◽

2015 ◽

Vol 2015 ◽

pp. 1-6

Author(s):

S. Popeskou ◽

M. Gavillet ◽

N. Demartines ◽

D. Christoforidis

Keyword(s):

Bone Marrow ◽

Gastrointestinal Bleeding ◽

Blood Cells ◽

Hemophagocytic Lymphohistiocytosis ◽

Hemophagocytic Syndrome ◽

Mortality Rates ◽

Successful Outcome ◽

Intestinal Bleeding ◽

Activated Macrophages ◽

Medical Entity

This paper presents to the surgical community an unusual and often ignored cause of gastrointestinal bleeding. Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is a rare medical entity characterized by phagocytosis of red blood cells, leucocytes, platelets, and their precursors in the bone marrow by activated macrophages. When intestinal bleeding is present, the management is very challenging with extremely high mortality rates. Early diagnosis and treatment seem to be the most important factors for a successful outcome. We present two cases and review another 18 from the literature.

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The Hemophagocytic Syndrome In Prostate Cancer Revealed By Disseminated Carcinomatosis Of The Bone Marrow

The Journal of Urology ◽

10.1016/s0022-5347(05)64588-0 ◽

2002 ◽

Vol 168 (3) ◽

pp. 1101-1102 ◽

Cited By ~ 8

Author(s):

K. Koizumi ◽

Y. Haseyama ◽

R. Machino ◽

Y. Sato ◽

K. Sawada ◽

...

Keyword(s):

Prostate Cancer ◽

Bone Marrow ◽

Hemophagocytic Syndrome

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Autoimmune-Associated Hemophagocytosis and Myelofibrosis in a Newly Diagnosed Lupus Patient: Case Report and Literature Review

Case Reports in Hematology ◽

10.1155/2019/3879148 ◽

2019 ◽

Vol 2019 ◽

pp. 1-7

Author(s):

Deonne Thaddeus V. Gauiran ◽

Paula Victoria Catherine Y. Cheng ◽

Christopher Ryan P. Pagaduan ◽

Maria Clariza M. Santos

Keyword(s):

Bone Marrow ◽

Hemophagocytic Syndrome ◽

Target Organ Damage ◽

Organ Damage ◽

Target Organ ◽

High Dose ◽

Newly Diagnosed ◽

Timely Initiation ◽

Life Threatening ◽

Important Site

Bone marrow abnormalities in SLE are now becoming increasingly recognized, suggesting that the bone marrow may also be an important site of target organ damage. In this study, we present a rare case of concurrent autoimmune hemophagocytic syndrome and autoimmune myelofibrosis, potentially life-threatening conditions, in a newly diagnosed SLE patient. We report a case of a 30-year-old Filipino woman who presented with a one-year history of fever, constitutional symptoms, exertional dyspnea, joint pains, and alopecia and physical examination findings of fever, facial flushing, cervical lymphadenopathies, and knee joint effusions. Laboratory workup revealed pancytopenia with leukoerythroblastosis, elevated ESR, increased serum levels of transaminases, elevated CRP and LDH, hyperferritinemia, hypertriglyceridemia, proteinuria, hepatomegaly, and positive antinuclear antibody. Bone marrow aspiration and trephine biopsy revealed hemophagocytosis and moderate myelofibrosis. The patient was diagnosed with SLE with concomitant autoimmune-associated hemophagocytic syndrome and autoimmune myelofibrosis. Treatment with high-dose corticosteroids led to dramatic clinical improvement with normalization of laboratory data and complete resolution of bone marrow hemophagocytosis and myelofibrosis. Hemophagocytosis and myelofibrosis, although uncommon, are possible initial manifestations of SLE and should be included in the differential diagnosis of cytopenias in SLE. Thorough clinical assessment and microscopic bone marrow examination and timely initiation of corticosteroid therapy are essential in the diagnosis and management of these potentially life-threatening conditions. This case emphasizes that the bone marrow is an important site of target organ damage in SLE, and evaluation of cytopenias in SLE should take this into consideration.

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Clinical Characteristics of T-Cell Lymphoma Associated Hemophagocytic Syndrome: Comparison of T-Cell Lymphoma with and without Hemophagocytic Syndrome.

Blood ◽

10.1182/blood.v110.11.3576.3576 ◽

2007 ◽

Vol 110 (11) ◽

pp. 3576-3576

Author(s):

Hongyan Tong ◽

Yanling Ren ◽

Feng Xiao ◽

Wenyuan Mai ◽

Haitao Meng ◽

...

Keyword(s):

Bone Marrow ◽

T Cell ◽

Median Survival ◽

Clinical Characteristics ◽

Hemophagocytic Syndrome ◽

Cell Lymphoma ◽

Initial Therapy ◽

T Cell Lymphoma ◽

Β2 Microglobulin ◽

Significant Difference

Abstract T-cell lymphoma associated hemophagocytic syndrome (T-LAHS) has been regularly reported in Asia countries and is considered with extremely poor prognosis. The rate of definite diagnosis during early stage is low and the therapeutic outcome has been disappointed. We therefore compared T-cell lymphoma patients with and without hemophagocytic syndrome (HPS) in order to have a better understanding of the clinical characteristics of T-LAHS. One hundred and thirteen patients (66 men and 47 women, age from 12 to 80 years with the median age of 42) with aggressive T-cell lymphoma admitted to our department between January 2000 and December 2005 were included in this study, while 28 of them were with T-LAHS. The patients were divided into LAHS group and no-LAHS group. The clinical data including clinical manifestations and laboratory findings were compared between the two groups by using Chi-square test. The method of Kaplan and Meier was used to analyze overall survival (OS). The results showed that LAHS occured in about 1/4 of all the patients with T-cell lymphoma, which were all aggressive type. The elevated rates of lactate dehydrogenase (LDH) and ferritin were much higher in LAHS group than in no-LAHS group. β2-microglobulin and ovarian cancer antigen (CA125) were also elevated in both groups, but there was no significant difference. The rate of hypo-fibrinogen and liver dysfunction were higher in LAHS group than that in no-LAHS group. The rate of bone marrow infiltration in LAHS group is remarkably higher than that in no-LAHS group (57% vs 32%, p<0.05). The median survival was 40 days (16 days - 22 months) in the LAHS group, and the median survival of 11 patients accepted chemotherapy more than 2 courses was 6 months. By contrast, the 2-year survival for no-LAHS group was 43%. There was significant difference between the two groups. Three patients undergoing plasmapheresis as initial therapy had survived for 3–6 months. These results indicate that high suspicion is required for early diagnosis of T-LAHS. In patients with fever, hepatosplenomegaly and cytopenia, simultaneously with serum markers such as LDH, ferritin, TG, CA125, and β2-microglobulin constantly increasing, T-LAHS should be considered. For patients without extranodal invasion or enlargement of lymph nodes, repeating biopsy of multiple sites of bone marrow may help improving the diagnosis rate. As for treatment, other more intensive regimens were not superior to CHOP regimen. While the overall outcome of treatment is still unsatisfied, plasmapheresis as initial therapy is worth considering.

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Human Granulocytic Anaplasmosis: a Case Report and Review of Literature.

Blood ◽

10.1182/blood.v114.22.4518.4518 ◽

2009 ◽

Vol 114 (22) ◽

pp. 4518-4518

Author(s):

Jian Ouyang ◽

Qiguo Zhang ◽

Jingyan Xu ◽

Bing Chen ◽

Rong-Fu Zhou

Keyword(s):

Bone Marrow ◽

Hemophagocytic Syndrome ◽

Plasma Cells ◽

Conflicts Of Interest ◽

Diagnosis And Treatment ◽

Case Presentation ◽

Human Granulocytic Anaplasmosis ◽

Granulocytic Anaplasmosis ◽

Laboratory Features ◽

Bone Marrow Plasma

Abstract Abstract 4518 Purpose To discuss the clinical and laboratory features, diagnosis and treatment of human granulocytic anaplasmosis. Methods We present the clinical and laboratory features, diagnosis and treatment of a patient with human granulocytic anaplasmosis. Case presentation The patient with human granulocytic anaplasmosis presented with fever,cough?Adiarrhea?Amyalgia?Afacies typhosa?Arelative infrequent pulse and swelling of lymph nodes. Laboratory examination showed the patient had leukopenia?Athrombocytopenia?Aproteinuria?Aliver injury?Ablood clotting abnormal?AEBV-DNA positive. We also found the patient's ferritin?Acreatase?Aamylase and lipase increased. In the patient's bone marrow, plasma cells were increased, hemophagocyte and intragranulocytic inclusions were found. The patient did not respond to the treatment of imipenem, cefepime hydrochloride and teicoplanin. But he was treated successfully with moxifloxacin. Conclusion Patient with human granulocytic anaplasmosis can present leukopenia, thrombocytopenia, blood clotting abnormal and plasma cells increased in bone marrow. It's quite necessary to make differential diagnosis with some blood diseases. The patient can be accompanied with EBV infection and hemophagocytic syndrome. The patient can be cured by antibiotics-quinolones. Disclosures: No relevant conflicts of interest to declare.

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