Juvenile Myelomonocytic Leukemia

Juvenile myelomonocytic leukemia (JMML) is a rare fatal hematopoietic disorder of early childhood. We are presenting a case of 9-month-old female child who was admitted with abdominal distension, irritability, and hepatosplenomegaly. Peripheral blood film examination showed leukoerythroblastosis with leukocytosis, absolute monocytosis, microcytic hypo chromic anemia, and thrombocytopenia. Bone marrow examination showed myeloid hyperplasia, Hb HPLC revealed normal HbF (1.3 %) and HbA2 (2.9 %). There was absolute gamma globulinemia and DCT positivity. Cytogenetic studies revealed a normal karyotype with absence of Philadelphia (Ph) chromosome, monosomy 7 or any other chromosomal abnormality. Diagnosis of JMML was rendered according to the diagnostic criteria laid down by WHO classification 2008 with presence of peripheral blood monocytosis >1 × 10⁹/L, blasts <20 % of leucocytes in blood or nucleated cells in bone marrow, absence of Ph chromosome, presence of immature granulocytes in the blood and WBC count >10 × 10⁹/L. The patient was then started on a regimen of chemotherapy to which she gave a promising response.     

Juvenile myelomonocytic leukemia characterized by cutaneous lesion containing Langerhans cell histiocytosis-like cells

We present a 1-year-old boy who developed a cutaneous lesion on the trunk and hepatosplenomegaly. Laboratory examination showed leukocytosis with peripheral blasts, atypical monocytosis, anemia, hyper IgG, and a mild elevation of C-reactive protein. Clinical features and skin biopsy findings matched the diagnostic criteria of both juvenile myelomonocytic leukemia (JMML) and Langerhans cell histiocytosis (LCH). Histopathology revealed atypical mononuclear cells that had infiltrated around vessels throughout the dermis in a skin biopsy specimen. These cells were CD1a (+), S-100 (+), CD68 (+), CD207 (−), lysozyme (+), and myeloperoxidase (−). The diagnosis of JMML was confirmed by detection of spontaneous colony formation and granulocyte–macrophage colony-stimulating factor hypersensitivity in vitro, and a somatic NRAS point mutation. Transplantation of bone marrow from an HLA-matched unrelated donor was performed, and the marrow was successfully engrafted. The cutaneous lesion and hepatosplenomegaly were improved at the time of discharge. It is often difficult to distinguish between JMML and LCH-like infiltrates by assessing clinical and light microscopic features of various cutaneous lesions. In the current case, molecular biological analysis enabled us to develop a precise diagnosis.     

Eosinophilia during fludarabine treatment of chronic lymphocytic leukemia

 Although eosinophilia has been reported as a side effect of purine analogues, there is no report on fludarabine-induced eosinophilia in chronic lymphocytic leukemia (CLL). During chemotherapy with fludarabine and cyclophosphamide, we observed two cases of significant eosinophilia. A 67-year-old patient with CLL developed bone marrow and peripheral blood eosinophilia up to 7.9×10⁹/l, the highest eosinophil count ever reported during treatment with a purine analogue. The eosinophilia persisted for 33 days. Another patient developed bone marrow eosinophilia without eosinophilia in the peripheral blood. These are the first documented cases of fludarabine-induced eosinophilia in CLL, and this side effect may conceivably be more common than previously recognized. Received: January 12, 1999 / Accepted: April 28, 1999     

Impact of pre-induction therapy leukapheresis on treatment outcome in adult acute myelogenous leukemia presenting with hyperleukocytosis

9 /l were scheduled to undergo leukapheresis. This represented 53 patients (median age 59 years, range 16–78 years) who underwent from 1 to 4 sets of leukapheresis (median 1). The median initial WBC count was 160×10⁹/l (range 100–480×10⁹/l). Morphologic subtypes, according to the French–American–British classification, showed 3 M0, 16 M1, 6 M2, 10 M4, 16 M5, and 2 unclassified cases of AML. In 21 patients (40%), leukapheresis did not reduce their WBC counts significantly, while 32 patients (60%) achieved a WBC count of less than 100×10⁹/l (median 71×10⁹/l) after leukapheresis. Analysis of cell cycle was performed on bone marrow (BM) and peripheral blood leukemic cells before and after leukapheresis in three cases. In two of those cases, a recruitment of BM leukemic cells in the S phase was observed after leukapheresis. The median WBC count at the time of starting chemotherapy was 85×10⁹/l (range 23–264×10⁹/l). Complete remission was achieved in 55% (95% confidence interval 40–68%). Early death occurred in two cases. Median disease-free survival was 10 months, while median overall survival was 8 months. In this study, early death rate is lower than data previously published in the literature and almost all patients could receive chemotherapy. This might suggest a benefit of initial leukapheresis in the treatment of AML presenting with hyperleukocytosis. Received: 23 November 1999 / Accepted: 8 March 2000     

Bone marrow CD34+ progenitor cells from rheumatoid arthritis patients support spontaneous transformation of peripheral blood B cells from healthy individuals

We show that bone marrow (BM) CD34⁺ progenitor cells from rheumatoid arthritis (RA) patients have the capacity to support spontaneous transformation of peripheral blood B cells. CD34⁺ cells purified from BM blood from eight RA patients and eight osteoarthritis (OA) patients were expanded with granulocyte/macrophage colony stimulating factor (GM-CSF) for 4–6 weeks. GM-CSF-stimulated BM CD34⁺ cells from three of eight RA patients, but none from seven OA patients, gave rise to spontaneous transformation of highly purified B cells of Epstein-Barr virus (EBV)- seronegative healthy donors. GM-CSF-stimulated BM CD34⁺ cells from four of six RA patients and from one of four OA patients also supported the spontaneous transformation of peripheral blood B cells from EBV-seropositive healthy donors. All the transformed B cell lines were positive for EBV-DNA as determined by PCR. Neither GM-CSF-stimulated BM CD34⁺ cells alone nor highly purified B cells alone gave rise to spontaneously transformed B cell lines. These results suggest that the capacity of BM CD34⁺ cells to support survival of B cells might contribute to the pathogenesis of RA by sustaining abnormal B cell responses. Received: 8 November 1999 / Accepted: 10 January 2000     

Changes in bone marrow and peripheral blood lymphocyte subset findings with onset of hepatitis-associated aplastic anemia

Rationale:Hepatitis-associated aplastic anemia (HAAA) is a rare illness that results in bone marrow failure following hepatitis development. The etiological agent remains unknown in most HAAA cases. However, clinical features of the disease and immunotherapy response indicate that immune-mediated factors play a central role in the pathogenesis of HAAA. Activation of cytotoxic T cells and increase in CD8 cells could exert cytotoxic effects on the myelopoietic cells in the bone marrow.Patient concerns:A 15-month-old boy was brought to our hospital with complaints of generalized petechiae and purpura observed a week prior to hospitalization. His liver was palpated 3 cm below the costal margin, platelet count was 0 × 10⁴/μL, and alanine aminotransferase level was 1346 IU/L. A blood test indicated cytomegalovirus infection, and 3 bone marrow examinations revealed progressive HAAA. As the disease progressed to the 3^rd, 6^th, and 9^th week after onset, CD4⁺ T cells were markedly decreased, CD8⁺ T cells were markedly increased, and the CD4/CD8 ratio was significantly decreased. The number of B cells and natural killer cells decreased with time, eventually reaching 0.0%.Diagnosis:HAAA.Interventions:Rabbit antithymocyte globulin and eltrombopag olamine (a thrombopoietin receptor agonist) were administered.Outcomes:The patient''s platelet count returned to normal, and bone marrow transplantation was avoided. The peripheral blood lymphocytes (PBLs) improved as the patient''s general condition recovered.Lessons:This case demonstrates that HAAA induced by cytomegalovirus infection features decreasing CD4⁺ and increasing CD8⁺ PBLs as the bone marrow hypoplasia progresses. The PBLs return to their normal levels with the recovery from the disease. Our case findings thus support the involvement of immunological abnormality in HAAA.     

IgE γ paraproteinaemia associated with a pleomorphic lymphoproliferative disorder

Summary A new case of IgEγ paraproteinaemia is described in which the immunoproliferative disorder was lympho-plasmacytoid, rather than myeloma-tous in type. The patient, an 81-year-old woman, lacked both organomegaly and bone lesions. The peripheral white blood count was 30 × 10⁹/l (43% lymphocytes and 37% lymphoplasmacytoid cells) and the bone marrow was heavily infiltrated with mononuclear cells with 50% lymphocytes and 50% lymphoplasmacytoid cells). Detailed immunological marker analysis of both peripheral blood and marrow showed an increased population of morphologically normal T cells, together with a neoplastic population of B cells. The neoplastic cells were shown to re-express surface immunoglobulin after stripping with anti-E anti-serum, and to synthesis Ig in radio-incorporation experiments. The immunological characteristics of the neoplastic cells are considered in relation to B-cell maturation and it is shown that the immunological phenotype was of the intermediate type to be expected in association with a lympho-plasmacytoid proliferation.     

B-cell lymphoma-associated hemophagocytic syndrome: clinicopathological characteristics

Seven patients with peripheral B-cell lymphoma associated with hemophagocytic syndrome are reported. In all cases, the histologic subtype was diffuse large B-cell lymphoma. Hemophagocytic features were noted in the bone marrow with lymphomatous infiltration. Hemophagocytic syndrome occurred with presentation of the lymphoma and was characterized by high fever, cytopenias, and elevated levels of lactate dehydrogenase, ferritin, C-reactive protein, and cytokines [interferon γ, macrophage colony-stimulating factor, soluble interleukin (sIL)-2R, and IL-6] without evidence of infection. The phenotypes of lymphomas were suspected CD19⁺, CD20⁺, S-Ig⁺, CD10⁻, and co-expression of CD5 in some cases. Flow cytometric analysis showed a low CD4/CD8 ratio in peripheral blood and bone marrow. We suggest that the pathogenesis of hemophagocytic syndrome is hypercytokinemia induced by a proliferation of reactive CD8⁺ T cells. Previous reports of B-cell lymphoma with hemophagocytic syndrome demonstrated similar clinical manifestations and poor prognoses. The invasion patterns of these diffuse large B-cell lymphomas with hemophagocytosis may be classified into three groups: microscopic lymph-node involvement type, gross lymph-node involvement type, and splenic lymphoma type. Although hemophagocytic syndromes have been reported to be associated with T-cell lymphomas, our results indicate an association with diffuse large B-cell lymphoma. Received: 16 July 1999 / Accepted: 13 December 1999     

Changes of immunological markers after autologous peripheral blood stem cell transplantation

Recently high-dose chemotherapy with peripheral blood stem cell transplantation (PBSCT) has become an important treatment for hematological and solid tumors. Methods: Immunological parameters were examined before and after PBSCT in 9 patients with lung cancer and 13 patients with malignant lymphoma. Findings were compared with those for bone marrow transplantation (BMT). Peripheral blood cells were analyzed for phenotype and the levels of cytokines and soluble factors were measured. Results: After PBSCT, activated T cells (CD3⁺HLA-DR⁺ cells, CD8⁺HLA-DR⁺ cells) and suppressor/cytotoxic T cells (CD8⁺CD11b⁻ cells) were significantly higher in the patients with lung cancer than in those with malignant lymphoma. Serum levels of interleukin-4 and soluble interleukin-2 receptor were also significantly higher in the patients with lung cancer than in those with lymphoma. On the other hand, the serum levels of interferon γ, tumor necrosis factor α, interleukin-6, soluble human leukocyte antigen class 1, and soluble thrombomodulin were significantly increased after bone marrow transplantation. The transfused peripheral stem cells of lung cancer and lymphoma patients had a similar number of granulocyte/macrophage-colony-forming units, but lung cancer patients had significantly more CD34-positive cells. Conclusion: By reinfusing large numbers of autologous immune cells, PBSCT may accelerate immune reconstitution, with T cells being likely to have a marked therapeutic potential. The changes after PBSCT were greater in patients with lung cancer than in lymphoma patients. These blood cells are potent mediators of anticancer activity and could play an important role in the elimination of autologous malignant cells. Received: 20 April 1998 / Accepted: 24 August 1998     

Juvenile myelomonocytic leukemia (JMML) with the hematologic phenotype of severe β thalassemia

A 3-year-old Filipino-American child with recurrent fever, splenomegaly, anemia, and thrombocytopenia, was found to have a hemoglobin F level of 76.9%. His reticulocyte count was elevated (4.3%), and erythroblasts were present in his peripheral blood. The child's erythrocytes were microcytic (MCV 66.9 fl) but his serum ferritin level was normal. His bone marrow at initial presentation demonstrated normal cellularity without an increase in blast cells. The disease progressed with worsening anemia, leukocytosis, and thrombocytopenia, with increased blasts in his marrow and the appearance of a mediastinal mass. His liver, spleen, and lymph nodes were found to be infiltrated with myeloblasts, supporting a diagnosis of juvenile myelomonocytic leukemia (JMML). Analysis of the child's Hb F showed a ^Gγ/^Aγ ratio of 2.2, which was within the characteristic range for JMML. A globin synthesis study using blood reticulocytes showed an α/non-α globin synthesis ratio of 2.24, typical of severe homozygous β thalassemia. Southern blot analysis of blood-leukocyte DNA from the patient and his parents demonstrated no apparent abnormality in the β-globin gene promoter or coding regions. The elevated level of Hb F in this child with JMML appeared to be part of an acquired Cooley's anemia-like hematologic phenotype. Am. J. Hematol. 58:67–71, 1998. © 1998 Wiley-Liss, Inc.     

In vitro steroid sensitivity testing: A possible means to predict response to therapy in primary hypoproliferative anemia

We investigated the effects of various steroids on erythroid colony formation by normal human bone marrow and peripheral blood, and by marrow and peripheral blood from 18 patients with primary hypoproliferative anemia. These agents were variously found to enhance both CFU-E and BFU-E derived colony growth by normal human cells. Fluoxymesterone and dexamethasone were the most active inducers of CFU-E proliferation, and etiocholanolone and dexamethasone were the most potent burst augmenters. Androsterone did not significantly influence BFU-E proliferation in 66% of the marrow cultures from hematologically normal donors. Colony formation by erythroid progenitor cells of the patients with hypoproliferative anemia was reduce (20 ± 10 CFU-E derived colonies/6 × 10⁴ marrow cells; 12 ± 5 BFU-E derived colonies/1 × 10⁵ blood cells) when compared to growth by normal cells (65 ± 14 CFU-E derived colonies/6 × 10⁴ marrow cells; 21 ± 9 BFU-E derived colonies/1 × 10⁵ blood cells). Colony formation by marrow or peripheral blood cells of eight patients with steroid-responsive anemia was only moderately reduced (26 ± 11 CFU-E derived colonies/6 ± 10⁴ marrow cells; 17 ± 3 BFU-E derived colonies/1 × 10⁵ blood cells) when compared to growth by marrow cells of three steroid-unresponsive patients (3 ± 1.5 CFU-E derived colonies/6 × 10⁴ cells). Whereas the addition of steroids of the same class to marrow and peripheral blood cultures of the steroid-responsive patients enhanced colony growth by 60–300%, their addition to marrow cultures of the steroid-unresponsive patients increased colony growth by less than 60%. It appears that further investigations using in vitro culture techniques as predictors of response to steroid therapy in patients with hypoproliferative anemia may be warranted.     

Successful transplantation and engraftment of peripheral blood stem cells after cryopreservation, positive and negative purging procedures, and a second cryopreservation cycle

 Transplantation of peripheral blood stem cells (PBSC), positively and/or negatively selected immediately after harvest, has become a widely applied therapeutic option in hematological or oncological patients. The following case of peripheral blood stem cell transplantation represents the first case of successful transplantation of PBSC, cryopreserved twice and purged after cryopreservation. PBSC were harvested in a 44-year-old female patient with a low-grade non-Hodgkin's lymphoma stage IV after mobilization with chemotherapy and G-CSF. A total number of 15.2×10⁶ CD34⁺ cells/kg bodyweight was harvested with a 36.9% contamination of tumor cells coexpressing CD5 and CD20. After subsequent chemotherapy cycles and cyclophosphamide mobilization, only 0.77×10⁶ CD34⁺ cells/kg bodyweight, not sufficient for transplantation, were achieved after positive selection. Therefore, 10.8×10⁶ cryopreserved CD34⁺ cells/kg bodyweight were thawed and a positive selection was carried out with the BAXTER Isolex 300i machine. Before additional negative selection, the 0.77×10⁶ positively selected CD34⁺ cells/kg bodyweight from the second mobilization were added. A total quantity of 4.4×10⁶ CD34⁺ cells/kg bodyweight with a purity of 93.1% representing a recovery of 38% was obtained. Cells were again cryopreserved, stored and retransfused after conditioning the patient with TBI and high-dose cyclophosphamide. The patient engrafted with a WBC count >1000/μl on day eight and a platelet count >20,000/μl without transfusion support on day 12 post-transplantation. This case indicates that purging procedures can successfully be carried out with cryopreserved cell material and that purified CD34⁺ cells can be cryopreserved a second time before transplantation, without affecting their hematopoietic capacity. Received: 5 March 2000 / Accepted: 18 August 2000     

Primary cutaneous marginal center lymphoma – complete remission induced by interferon α2a

Marginal zone lymphoma (MZL) is a distinct entity among B-cell lymphomas. We report on a 53-year-old woman who developed disseminated primary cutaneous MZL with secondary lymph node involvement and perinodular spreading. The tumor cell phenotype was characterized as CD20/CD79a/κ/λ+/bcl-2-positive, CD3/5/15/39/bcl-1-negative. Ki-67 was expressed by 20–35% of tumor cells. There was no evidence of systemic (including bone marrow) involvement. The diagnosis of MZL with plasmacellular differentiation (Stage IVa) was made. The patient was treated with interferon α2a injected s.c. at 9 × 10⁶ U 3 days a week for 1 year. During this time the skin lesions completely disappeared. No evidence of lymph node or extracutaneous disease was found. The patient remains in complete remission. Side effects were only of grade I (WHO); the Karnovsky index was 90%. As shown for other types of primary cutaneous B-cell lymphoma, prolonged interferon α monotherapy may be effective in controlling the disease and/or inducing complete remission in MZL. Received: 11 December 1998 / Accepted: 13 January 1999     

E-selectin and very late activation antigen-4 mediate adhesion of hematopoietic progenitor cells to bone marrow endothelium

 Adhesion of CD34⁺ hematopoietic progenitor cells (HPCs) to sinusoidal endothelium probably plays a key role in homing of transplanted CD34⁺ HPCs to the bone marrow (BM). We have investigated the role of various adhesion molecules in the interaction of purified CD34⁺ HPCs derived from BM or peripheral blood (PB) and a human BM-derived endothelial cell line. Adhesion of CD34⁺ HPCs to endothelial cells was measured with the use of a double-color flow microfluorimetric adhesion assay. In this assay, adhesion is measured under stirring conditions, simulating blood flow in sinusoidal marrow vessels. Adhesion of PB CD34⁺ cells to human BM endothelial cells (HBMECs) was observed only after interleukin (IL)-1β prestimulation of the endothelial cells. This adhesion was strongly increased after addition of phorbol-myristate acetate (PMA). Adhesion of PB CD34⁺ cells to IL-1β-prestimulated HBMECs was inhibited by blocking monoclonal antibodies (mAbs) against E-selectin and by neuraminidase treatment of the PB CD34⁺ cells. mAbs against very late activation antigen (VLA)-4 inhibited adhesion only when the E-selectin-mediated interaction was prevented. No clear inhibiting effect was found with blocking mAbs against β₂-integrins. Stimulation with the β₁-integrin-activating mAb, 8A2, induced adhesion of CD34⁺ cells to endothelial cells. In conclusion, stimulation of both endothelial cells and CD34⁺ HPCs is necessary for adhesion of CD34⁺ HPCs to endothelial cells. We furthermore demonstrated that E-selectin and VLA-4 mediated this adhesion. Received: 26 April 1999 / Accepted: 8 February 2000     

Cerebellar myeloblastoma formation in CD7-positive, neural cell adhesion molecule (CD56)-positive acute myelogenous leukemia (M1)

 We present a first report of a CD7⁺ acute myelogenous leukemia patient who developed intracranial myeloblastomas. The patient was neurologically normal on physical examination at presentation. The peripheral leukocyte count was extremely high (203.6×10⁹/l). The blasts expressed CD7 and CD56 (neural cell adhesion molecule) in addition to CD13, CD33, CD34, and HLA-DR. The karyotype of bone marrow cells was normal. The patient was diagnosed as having acute myelogenous leukemia (AML, M1). Following a short period of complete remission, bone marrow relapse and meningeal leukemia occurred, and the patient died of respiratory failure. Autopsy revealed that blasts had invaded the subarachnoid space and cerebellum, and two myeloblastomas were found in the cerebellar hemisphere. Both CD7⁺ and CD56⁺ AML have been reported to have a high incidence of central nervous system involvement. CD7⁺ CD56⁺ AML calls for prophylaxis of central nervous system leukemia. Received: 2 May 1997 / Accepted: 17 July 1997     

Granulocyte and monocyte apheresis suppresses symptoms of rheumatoid arthritis: a pilot study

To investigate if granulocyte and monocyte apheresis mitigates the symptoms of rheumatoid arthritis (RA), and influences production of panmyelocytes (CD15⁺ CD16^– cells) at the bone marrow level, 27 RA patients who had elevated granulocyte counts were recruited. The granulocyte and monocyte apheresis column (G-1 column) is an extracorporeal type device packed with 220 g cellulose acetate beads to which granulocytes and monocytes specifically adhere. Patients received apheresis of 1 hr duration twice per week, 8 times over a period of 4 weeks. To prepare CD15⁺CD16^– cells, iliac bone marrow aspirate was obtained at baseline and at 2 weeks after completion of the apheresis course. Ex-vivo proliferation of bone marrow low density cells and production of IgM-RF were also investigated. Following granulocyte and monocyte apheresis, there was a suppressed tendency in the number of CD15⁺CD16^– cells in patients with high bone marrow CD15⁺CD16^– cell counts at baseline. Clinical assessments 2 weeks after the completion of apheresis therapy showed improvements in swollen joint count (P<0.001), tender joint count (P<0.001) and duration of morning stiffness (P<0.005). The results suggest that granulocytes and monocytes/macrophages have a pathological role in RA and apheresis treatment to reduce or suppress these cells should benefit patients with RA. Received: 23 December 1997 / Accepted: 27 July 1998     

Refractory anemia with ringed sideroblasts associated with thrombocytosis: comparative analysis of marked with non-marked thrombocytosis, and relationship with JAK2 V617F mutational status

The World Health Organization (WHO) classification of tumors of hematopoietic and lymphoid tissues (2001) defined a provisional entity named refractory anemia with ringed sideroblasts associated to marked thrombocytosis (RARS-MT). Diagnosis of RARS-MT requires more than 15% of ringed sideroblasts in bone marrow aspirate and the existence of a thrombocytosis in blood, with a platelet count above 600 × 10⁹/L. Nevertheless, controversy exists regarding this platelet count “cut-off” value and, when RARS-MT was defined, the JAK2 mutation and its importance in the study of myeloproliferative disorders was unknown. We present the results of a Spanish retrospective multicentric study, which includes 76 cases of RARS with associated thrombocytosis (platelet count above 400 × 10⁹/L) at diagnosis (RARS-T), 36 of them with a platelet count above 600 × 10⁹/L. Our aim was to analyze their clinical, analytical and morphological characteristics, and to establish correlations with the JAK2 mutational status. This work is multicentric, with data collected and centralized at Hospital Universitario de Canarias (La Laguna, Spain).     

F-18 FDG uptake of bone marrow on PET/CT scan: it’s correlation with CD38/CD138 expressing myeloma cells in bone marrow of patients with multiple myeloma

The percentage of myeloma cells in bone marrow is subsequently an important index of disease in patients with multiple myeloma (MM). Bone marrow myeloma cells can be detected by strong CD38/CD138 positivity and light scatter characteristics using flow cytometry. The aim of the study was to evaluate the relationship between the degree of F-18 fluorodeoxyglucose (F-18 FDG) uptake and the percentage of CD38/CD138 expressing myeloma cells in the bone marrow of patients with MM. A total of 31 patients with MM (14 females and 17 males, mean age 59.5 ± 1.9 years, range 29–80 years) were included in the study. All patients underwent FDG-positron emission tomography/computed tomography (PET/CT) scan within 2 weeks after the completion of the usual staging workup for MM, consisting of X-ray skeletal survey and hematological/biochemical parameters including complete blood count, liver and kidney function test, erythrocyte sedimentation rate, serum immunoglobulins, urine light chain excretion, C-reactive protein, β2-microglobulin, and bone marrow plasma cell infiltration. In all patients, flow cytometry was performed for assessing the percentage of CD38/CD138 expressing myeloma cells in the bone marrow samples. The extent of bone marrow FDG uptake on PET/CT scans was visually graduated using a qualitative scoring system as extension score (E-score) and also a semiquantitative scoring system defined as mean standardized uptake value (mSUV) of both femora. There was a statistically significant positive correlation between the percentage of CD38/CD138 expressing plasma cells in bone marrow and both mean qualitative (r = 0.616) and semiquantitative (r = 0.755) results of F-18 FDG uptakes. mSUV and E-score of bone marrow FDG uptake values were also correlated with serum beta-2-microglobulin levels (r = 0.523 and r = 479, respectively). mSUV of bone marrow FDG uptake values were also negatively correlated with serum albumin levels (r = −0.424), whereas there was no correlation between E-score and albumin levels. In conclusion, our results indicate that increased F-18 FDG uptake of bone marrow is related to the percentage of plasma cell infiltration of bone marrow in patients with MM. Therefore, F-18 FDG-PET/CT study may be a useful tool for predicting the levels of myeloma cells in bone marrow, and an additional analysis of FDG uptake of bone marrow on FDG-PET/CT scans should be performed in patients undergoing PET studies during the initial staging, evaluating the therapy response, and monitoring patients with MM.     

The management of isolated thrombocytopenia in Chinese adults: does bone marrow examination have a role at presentation?

We performed a retrospective analysis of bone marrow examination (BME) in the management of Chinese adult patients less than 60 years of age with isolated thrombocytopenia at presentation. Eighty‐three patients with a median age of 39 years presenting with isolated thrombocytopenia (median platelet count: 38 × 10⁹/l) had routinely undergone BME as part of the laboratory investigations during the period from January 1996 to December 1999. All 83 patients had bone marrow findings of active marrow suggesting causes due to peripheral destruction. All of these patients responded to steroid or intravenous immunoglobulin (IVIg) therapy at presentation if their platelet counts were significantly low or if they had mucosal bleeding. Eighty‐one of the 83 patients, after a median of 20 months follow‐up, were finally diagnosed as having idiopathic thrombocytopenic purpura (ITP). The remaining two patients were finally confirmed as cases of systemic lupus erythematosus (SLE). Our results suggest that BME is not helpful in the diagnosis of isolated thrombocytopenia or suspected ITP in adult patients at presentation, provided that a thorough clinical history and physical examination are undertaken and that the blood count and peripheral blood smear show no abnormalities apart from the thrombocytopenia.