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Acute myelocytic leukemia in a patient with hemophagocytic lymphohistiocytosis: A case report.

Zhao D, Qian L, Shen J - Oncol Lett (2014)

Bottom Line: The present study reports a rare case of this syndrome in combination with acute myeloblastic leukemia (AML-M2), in a patient with clonal karyotypic abnormalities.The patient was successfully treated with chemotherapy comprising daunorubicin (40 mg/m(2) i.v., days 1-3) and cytosine arabinoside (100 mg/m(2), 1-h i.v. infusion, days 1-7).All clinical symptoms disappeared following chemotherapy.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, Navy General Hospital, Beijing 100048, P.R. China.

ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is an aggressive hyperinflammatory condition characterized by prolonged fever, cytopenias and hepatosplenomegaly, as well as hemophagocytosis by activated, morphologically benign macrophages. HLH may be characterized into two forms, familial and secondary HLH. Familial HLH usually manifests in children with genetic abnormalities associated with the cytotoxic function of NK and T cells, whereas secondary HLH usually occurs in older patients in combination with an associated condition, such as infection or malignancy, without an identifiable genetic abnormality. Malignancy-associated hemophagocytic lymphohistiocytosis is mostly accompanied by lymphoid neoplasms. The present study reports a rare case of this syndrome in combination with acute myeloblastic leukemia (AML-M2), in a patient with clonal karyotypic abnormalities. The patient was successfully treated with chemotherapy comprising daunorubicin (40 mg/m(2) i.v., days 1-3) and cytosine arabinoside (100 mg/m(2), 1-h i.v. infusion, days 1-7). All clinical symptoms disappeared following chemotherapy.

No MeSH data available.


Related in: MedlinePlus

Bone marrow aspirate smear showed phagocytosis at the diagnosis of hemophagocytic lymphohistiocytosis (stain, hematoxylin and eosin; magnification, ×1,000).
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f1-ol-08-06-2634: Bone marrow aspirate smear showed phagocytosis at the diagnosis of hemophagocytic lymphohistiocytosis (stain, hematoxylin and eosin; magnification, ×1,000).

Mentions: A 61-year-old female presented to the Hematological Department of Navy General Hospital (Beijing, China) with a history of high fever for 30 days. The complete blood count showed pancytopenia; the white blood cell (WBC) count was 1.92×109/l (normal range, 4–10×109/l), the hemoglobin (Hgb) levels were 60 g/l (normal range, 120–150 g/l)and the platelet (PLT) count was 33×109/l (normal range, 100–300×109/l). Elevated levels of serum ferritin (11,966.7 μg/l; normal range, 11–360 μg/l) were detected. A reduced level of fibrinogen (Fg; 0.87 g/l; normal range, 1.5–4.0 g/l) and increased levels of lactate dehydrogenase (LDH; 1,617 U/l; normal range, 230–460 U/l), triglycerides (2.28 mmol/l; normal range, 1.7–2.25 mmol/l) and D-Dimer (6,779 μg/l; normal range, 0–300 μg/l) were detected. Serum antibody to Epstein-Barr virus (EBV) were negative, while serum antibodies to human immunodeficiency virus (HIV), hepatitis A, B and C (HAV, HBV and HCV), tubercle bacillus and hemococcidium were negative. Repeated blood cultures were negative. Bone marrow (BM) aspirate showed increased histiocytes (4.5%) with hemophagocytosis, and dysplasia in granulocytic and erythroid lineage. BM examination revealed a hypercellular marrow with 29% blasts accompanied by histiocytes with hemophagocytosis, but the erythroid progenitors were only 0.5% of total nucleated cells (Fig. 1). Morphology and flow cytometry studies showed no evidence of hematological malignancy. The levels of serum soluble interleukin 2 (IL-2) receptor (sCD25) in plasma of the BM were 44,000 pg/ml (normal levels, <6,400 pg/ml), while natural killer (NK) cell activity was 6.72% (normal range, 31.54–41.58%). G-banding analysis showed that these blasts had a chromosomal abnormality with 48,X, add(X)(p11),+3,der(7)t(1;7)(q11;p22),inv(12)(q15q24),add(14)(q32),t(14;19)(q32;q13),+18,add(21)(q22. CT scan examination revealed splenomegaly.


Acute myelocytic leukemia in a patient with hemophagocytic lymphohistiocytosis: A case report.

Zhao D, Qian L, Shen J - Oncol Lett (2014)

Bone marrow aspirate smear showed phagocytosis at the diagnosis of hemophagocytic lymphohistiocytosis (stain, hematoxylin and eosin; magnification, ×1,000).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4214506&req=5

f1-ol-08-06-2634: Bone marrow aspirate smear showed phagocytosis at the diagnosis of hemophagocytic lymphohistiocytosis (stain, hematoxylin and eosin; magnification, ×1,000).
Mentions: A 61-year-old female presented to the Hematological Department of Navy General Hospital (Beijing, China) with a history of high fever for 30 days. The complete blood count showed pancytopenia; the white blood cell (WBC) count was 1.92×109/l (normal range, 4–10×109/l), the hemoglobin (Hgb) levels were 60 g/l (normal range, 120–150 g/l)and the platelet (PLT) count was 33×109/l (normal range, 100–300×109/l). Elevated levels of serum ferritin (11,966.7 μg/l; normal range, 11–360 μg/l) were detected. A reduced level of fibrinogen (Fg; 0.87 g/l; normal range, 1.5–4.0 g/l) and increased levels of lactate dehydrogenase (LDH; 1,617 U/l; normal range, 230–460 U/l), triglycerides (2.28 mmol/l; normal range, 1.7–2.25 mmol/l) and D-Dimer (6,779 μg/l; normal range, 0–300 μg/l) were detected. Serum antibody to Epstein-Barr virus (EBV) were negative, while serum antibodies to human immunodeficiency virus (HIV), hepatitis A, B and C (HAV, HBV and HCV), tubercle bacillus and hemococcidium were negative. Repeated blood cultures were negative. Bone marrow (BM) aspirate showed increased histiocytes (4.5%) with hemophagocytosis, and dysplasia in granulocytic and erythroid lineage. BM examination revealed a hypercellular marrow with 29% blasts accompanied by histiocytes with hemophagocytosis, but the erythroid progenitors were only 0.5% of total nucleated cells (Fig. 1). Morphology and flow cytometry studies showed no evidence of hematological malignancy. The levels of serum soluble interleukin 2 (IL-2) receptor (sCD25) in plasma of the BM were 44,000 pg/ml (normal levels, <6,400 pg/ml), while natural killer (NK) cell activity was 6.72% (normal range, 31.54–41.58%). G-banding analysis showed that these blasts had a chromosomal abnormality with 48,X, add(X)(p11),+3,der(7)t(1;7)(q11;p22),inv(12)(q15q24),add(14)(q32),t(14;19)(q32;q13),+18,add(21)(q22. CT scan examination revealed splenomegaly.

Bottom Line: The present study reports a rare case of this syndrome in combination with acute myeloblastic leukemia (AML-M2), in a patient with clonal karyotypic abnormalities.The patient was successfully treated with chemotherapy comprising daunorubicin (40 mg/m(2) i.v., days 1-3) and cytosine arabinoside (100 mg/m(2), 1-h i.v. infusion, days 1-7).All clinical symptoms disappeared following chemotherapy.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, Navy General Hospital, Beijing 100048, P.R. China.

ABSTRACT
Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome, is an aggressive hyperinflammatory condition characterized by prolonged fever, cytopenias and hepatosplenomegaly, as well as hemophagocytosis by activated, morphologically benign macrophages. HLH may be characterized into two forms, familial and secondary HLH. Familial HLH usually manifests in children with genetic abnormalities associated with the cytotoxic function of NK and T cells, whereas secondary HLH usually occurs in older patients in combination with an associated condition, such as infection or malignancy, without an identifiable genetic abnormality. Malignancy-associated hemophagocytic lymphohistiocytosis is mostly accompanied by lymphoid neoplasms. The present study reports a rare case of this syndrome in combination with acute myeloblastic leukemia (AML-M2), in a patient with clonal karyotypic abnormalities. The patient was successfully treated with chemotherapy comprising daunorubicin (40 mg/m(2) i.v., days 1-3) and cytosine arabinoside (100 mg/m(2), 1-h i.v. infusion, days 1-7). All clinical symptoms disappeared following chemotherapy.

No MeSH data available.


Related in: MedlinePlus