All patients exhibited cytopenias
during their stay, although cell counts often fluctuated markedly, most commonly following transfusion of blood products.
Signs and symptoms include hepatosplenomegaly, prolonged fever, cytopenias
, elevated triglycerides and elevated ferritin.
5 2 factor Bone marrow <5 5--10 NA 11-- 21--30 blasts (%) 20 Karyotype Good Intermediate Poor NA NA Cytopenias
0/1 2/3 NA NA NA Median survival by age group (years) IPSS [greater >60 [greater than >70 classification than or (n=611) or equal (n=371) equal to]70(n=445) to]60 (n=205) Low (n=267) 11.
HLH should be ruled out in any previously healthy child that abruptly develops fever, hepatosplenomegaly, cytopenia
, and jaundice.
5[degrees]C; hypertriglyceridemia at 267 mg/dL (analysis performed at day 7); and cytopenias
, which preferentially affected erythrocytes and lymphocytes.
Hematological manifestations are usually asymptomatic; however, serious cytopenias
can develop in some cases, necessitating treatment.
Serum copper should also be measured in patients with unexplained cytopenia
or a suspected myelodysplastic syndrome.
The development of cytopenias
is an important feature of this syndrome.
In another Swedish study16 on 117 children with ALL during the maintenance treatment B19 DNA was found in 15 per cent cases with increased number of complications like cytopenia
causing significantly longer periods of unwanted interruptions of chemotherapy besides higher number of blood transfusions.
In conclusion, although the criteria of familial HLH were not appropriate for IAHS, in patients with infection and progression of hepatosplenomegaly and cytopenia
, the diagnosis of IAHS should be kept in mind.
In conclusion despite the very low frequency of secondary hematological malignancies in the setting of childhood acute lymphoblastic leukemia and lymphoblastic NHL the clinician should always be aware of this possibility when unexplained cytopenia
or leukocytosis occurs without evidence of a relapse of the primary disease.
The history is usually readily available and patients often have cytopenia
in other cell lines also.