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A 36-year-old man with fever and pancytopenia due to Hemophagocytic Lymphohistiocytosis is reported. The patient was started on the HLH-94 based treatment. Two weeks after the initiation of therapy the patient's pancytopenia had resolved and he was discharged to complete treatment as an outpatient. The initial clinical presentation, diagnostic criteria, pathophysiology and treatment will be discussed.

We describe a patient with typical hemophagocytic syndrome (HPS) in whom pancytopenia was refractory to steroid pulse therapy. He was successfully treated with immunosuppressive therapy using antithymocyte globulin (ATG) and cyclosporine (CyA), which is known to be effective for aplastic anemia (AA). Activation of histiocytes occurs in HPS as a response to several cytokines produced by activated T lymphocytes, while apoptosis of hematopoietic stem cells in AA is caused by T lymphocyte-derived cytokines. The response of this patient indicated that both diseases may have some similar immune-mediated conditions involving the activation of T lymphocytes and that intensive immunosuppressive therapy with ATG and CyA might be a useful strategy for steroid-resistant HPS.

Pancytopenia in an acutely ill child is commonly a result of bone marrow suppression. Rarely pancytopenia is a manifestation of inappropriate macrophage activation associated with hemophagocytosis. Viral infections account for most cases of secondary hemophagocytosis. We report a case of malaria-associated hemophagocytosis in a child from an endemic area. Systemic parasitic infections should be included in the differential diagnosis of pancytopenia and infection-associated hemophagocytosis. In this rare subgroup of hemophagocytosis, malaria caused by Plasmodium falciparum is the most common parasitic infection

Griscelli syndrome (GS) is a rare inherited disease characterized by immunodeficiency and partial albinism. The microscopic findings of the skin and hair are highly suggestive of the disease. The GS locus colocalizes on chromosome 15q21 with the myosin-Va gene (MYO5a), and mutations have been identified in few patients. We describe a 2-month-old Hispanic girl with severe pancytopenia secondary to hemophagocytosis. Even though a mutation at the Griscelli locus had not been identified, her clinical features and outcome were typical of GS. The purpose of this article is to alert physicians to the association between GS and hemophagocytosis. We suggest that GS should be considered in infants with hemophagocytosis because the features of partial albinism can be subtle. The relevant literature is summarized.

Case Report: We present the case of a feverish 36-year-old female patient with unspecific pain and weight loss. After initial diagnosis of an unclear infection with Candida esophagitis, unspecific duodenitis, proctitis and liver damage there was a further clinical deterioration during antibiotic and symptomatic therapy. A newly developed pancytopenia could be identified as caused by reactive hemophagocytosis using bone marrow analysis. The patient died despite maximal supportive and specific therapy. Cause of death was a retroperitoneal hematoma and an invasive aspergillosis, the latter not identified premortem., Conclusion: The reactive infect-associated hemophagocytosis is a rare differential diagnosis in feverish pancytopenia. The prognosis is poor. In absence of a proven causal therapy supportive therapy and maximal diagnostics for detection of curable underlying diseases are essential. Antimycotic therapy should be considered generally.

Hemophagocytic syndrome is an exceptional cause of pancytopenia. Its etiologies are most commonly viral or bacterial infections, lymphoproliferative syndromes, acquired or congenital immunodeficiencies, systemic diseases, or immunomodulatory treatment. We describe a patient with rheumatoid arthritis (RA) treated with methotrexate (MTX), sulfasalazine, and low dose corticosteroids, whose case was seriously complicated by the occurrence of acute febrile pancytopenia. The pancytopenia appeared secondary to hemophagocytic syndrome triggered by Escherichia coli septicemia. The evolution was marked by severe aggravation of RA, probably due to release of cytokines from macrophages (tumor necrosis factor-alpha, interleukin 6). Reintroduction of MTX (without sulfasalazine) resulted in partial remission and there was no reappearance of new hematological anomalies after 16 month followup. A knowledge of this syndrome is particularly important, since it mimics drug toxicity and other complications such as lymphoproliferative diseases.

Introduction: The aim of this work was to present a case of multicentric histiocytosis with haematologic involvement., Case Report: A 68-year-old man presented with poor general health and a nodular eruption of the skin and larynx. On clinical examination there was an enlarged spleen and laboratory results revealed an inflammatory syndrome, platelet count 60,000 and myelemia with 10 p. 100 immature elements in a white cell count of 14,000. Pathology and ultrastructure examinations led to the diagnosis of multicentric histiocytosis. Bone marrow aspiration was normal. Pancytopenia then developed with bone marrow hypoplasia without infiltration. Corticosteroids then cyclophosphamide were uneffective for either the skin disease or the hematologic disorder. The patient developed severe buccal aphthosis which responded well to thalidomide. This treatment reduced the size and the number of skin nodules. Finally, renal failure of unknown origin was observed., Discussion: Electron microscopy is essential for positive and differential diagnosis of atypical multicentric histiocytosis. Hematological disorders associated with multicentric histiocytosis may either be specific or totally independent.

We present a case of hemophagocytosis during infection with the intraërythrocytic protozoan Babesia microti in a 47-year-old splenectomized renal allograft recipient. After therapy with clindamycin and quinine a relapse responded to atovaquone; durable remission was not achieved until trimethoprim/sulfa was added. We postulate the severity of our patient's syndrome was due to splenectomy and chronic immunosuppression. Babesiosis should be considered when immunocompromised patients develop pancytopenia.

A 38-year-old black male is reported with a rare combination of disseminated tuberculosis together with a reactive histiocytic haemophagocytic syndrome and tuberculosis hypersplenism. Tuberculosis and histiocytic haemophagocytosis were diagnosed on bone marrow examination. The pancytopaenia and splenomegaly which were present on admission did not resolve despite adequate anti-tuberculosis chemotherapy. Prior to splenectomy the patient continued to have a marked thrombocytopenia which resulted in recurrent bouts of epistaxis; splenectomy together with tuberculostatic therapy was curative for the condition. The patient remains well with normal blood counts 1 year later.

Three cases of haemophagocytic syndrome (HPS) due to histoplasmosis in patients with the acquired immunodeficiency syndrome (AIDS) are described. At admission all had pancytopenia and the bone marrow aspirate showed typical haemophagocytosis and the presence of intracellular inclusions compatible with Histoplasma capsulatum. One patient had also cerebral toxoplasmosis. One patient died shortly after admission from multiple organ failure. Two patients survived, one treated with fluconazole and another with amphotericin-B with good response and progressive increase in their blood cell counts. We suggest that haemophagocytic syndrome due to disseminated histoplasmosis should be included in the causes of pancytopenia in patients with AIDS. Bone marrow aspirate is the best method for diagnosis.