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Biomedical subjects

U Göbel

Publications and source records attributed to U Göbel.

At least 19 recordsLinked to original sources
PubMed1999

Pelvic Ewing sarcoma: a retrospective analysis of 241 cases.

BACKGROUND: This article reports on 241 patients each with pelvic Ewing sarcoma registered for studies in Germany, Austria, and the Netherlands from January 1, 1981, until January 31, 1994. One hundred sixty-four patients had localized disease and 87 had metastases at diagnosis (PMP). Eighty-four patients with localized disease were entered on protocol (PP) and 80 were followed (FP). METHODS: Statistics included an analysis of event free survival by the Kaplan-Meier method and a Cox regression analysis of factors influencing prognosis. RESULTS: In the Kaplan-Meier analysis, on February 1, 1995, the event free survival (EFS) rate was 32% at 12 years for all patients, 54% for PP, 25% for FP, and 13% for PMP. Cox regression analysis showed that response to chemotherapy, initial metastases, and less intense therapy were significant prognostic factors. Among patients who had surgery for local control, the histologic response to chemotherapy was analyzed in the surgical specimen and had a significant influence on survival: EFS 69% for PP with good response compared with 47% (P = 0.11) for patients with poor response, and for FP 56% versus 13% (P = 0.002). All PP with small tumors had relapse free survival, compared with 69% of patients with medium-sized tumors and 36% of patients with tumors larger than 200 mL (P = 0.006). The initial tumor volume was a significant predictor of survival. CONCLUSIONS: Combined modality treatment has resulted in definitive improvement of prognosis for patients with localized pelvic Ewing sarcoma. However, the results for patients with metastases at diagnosis are still discouraging, and their treatment requires new approaches. Tumor load, responsiveness to chemotherapy, and adequate surgical margins are the major factors influencing the prognosis of patients with localized Ewing sarcoma of the pelvis.

Adolescent↗
PubMed1999

Evaluation of prognostic factors in a tumor volume-adapted treatment strategy for localized Ewing sarcoma of bone: the CESS 86 experience. Cooperative Ewing Sarcoma Study.

BACKGROUND: The Cooperative Ewing Sarcoma Study (CESS 86), conducted by the German Society of Pediatric Oncology and Hematology (GPOH), was planned on the basis of the results of the preceding CESS 81 study. The prognostic significance of tumor volume in localized Ewing sarcoma of bone was well documented in the CESS 81 trial. As a consequence, the treatment intensity was adapted to volume in the follow-up CESS 86 trial: the four-drug combination used in CESS 81 was amended for patients with large tumor volume (> or = 100 ml), where ifosfamide was substituted for cyclophosphamide. PROCEDURE: From January 1986 to June 1991, 177 protocol patients with localized Ewing sarcoma of bone were registered in CESS 86. The prognostic implication of tumor volume and several covariates was evaluated using Kaplan-Meier life table analysis and Cox's proportional hazard model. RESULTS: The estimated 5- and 8-year event-free survival (EFS) rates were both 59%. Age, gender, tumor site, and a tumor volume of 100 ml did not distinguish groups of patients with different prognosis. However, the prognosis of patients with tumors >200 ml (8-year EFS rate: 42%) was significantly inferior compared to patients with tumors both of 100 to 200 ml (70%) and of <100 ml (63%). In contrast to CESS 81, the histological response to chemotherapy was no longer a significant prognostic factor (EFS: 64% for good and 50% for poor responders, respectively). CONCLUSIONS: Despite risk-adapted treatment intensity, tumor volume retained its prognostic significance; the cut point, however, was shifted toward larger volumes.

Adolescent↗
PubMed1999

Monomorphic HLA class I-(non-A, non-B) expression on Ewing's tumor cell lines, modulation by TNF-alpha and IFN-gamma.

In this study, the expression of polymorphic and non-polymorphic MHC antigens in Ewing's tumor (ET) cells was examined by surface staining, Western blots and transcriptional analysis. Cell lines derived from Ewing's tumors largely lack polymorphic HLA class Ia antigens of both the HLA-A and the HLA-B loci but binding of monomorphic HLA antibodies indicates significant expression of HLA-C locus antigens and/or HLA class Ib molecules. HLA Ib molecules encoded by the HLA-E, -F or -G loci with a molecular mass of less than 44 kDa were not detected in lysates of either constitutive or TNF-alpha plus IFN-gamma treated ET cells. Two representative ET cell lines with either detectable HLA-A, -B antigens (A673) or absolutely non-detectable HLA-A, -B antigens (SK-ES-1) were further subjected to transcriptional analysis. A673 mRNA hybridized with HLA-A, -B, -C and HLA-E-specific probes in Northern blots. By contrast, mRNA specific for HLA-A, -B, -C was negative in SK-ES-1 but TNF-alpha plus IFN-gamma reconstituted HLA-A, -B, -C transcription in this cell line. HLA-E was transcribed in A673 but not in SK-ES-1. Combining mRNA and surface expression of HLA class Ia molecules results in a highly variable pattern of defective HLA class I expression in this type of neuroectodermal tumor. The involvement of the ET-specific fusion transcript EWS/Fli-1 in modulating the HLA-A and -B locus antigens is likely to occur by the upregulation of c-myc in these tumors. The exceptionally constant expression of HLA-C or some other non-A, non-B antigens (reactive with defined monoclonal antibodies) implies important consequences on tumor-cell resistance against specific CTL and NK activity in vivo.

Gene Expression↗
PubMed1998

Defective expression of granulocyte-macrophage colony-stimulating factor/interleukin-3/interleukin-5 receptor common beta chain in children with acute myeloid leukemia associated with respiratory failure.

Deficiency of the granulocyte-macrophage colony-stimulating factor (GM-CSF)/interleukin-3 (IL-3)/IL-5 receptors common beta chain (betac) is a cause of fatal respiratory failure. betac deficiency manifests as pulmonary alveolar proteinosis (PAP). PAP has heterogenous etiologies that may be genetic or aquired. Some cases of PAP have been reported to be associated with hematologic malignancies such as acute myeloid leukemia (AML). In mice, the PAP phenotype was generated by targeted deletion of the gene for betac and can be treated by transplantation of wild-type bone marrow into betac -/- mice. Thus, our findings in betac -/- mice provide evidence for a causal relationship between the lung disease and the hematopoietic system. We describe here expression defects of betac or betac plus GM-CSF receptor alpha chain (GM-CSFR alpha) in 3 pediatric patients with AML and PAP symptoms. All of the patients' leukemic cells failed to express normal levels of betac. The leukemic cells of patients no. 2 and 3 additionally lacked the expression of GM-CSFR alpha, as shown by flow cytometry. Strikingly reduced or absent function of betac was demonstrated in clonogenic progenitor assays with absent colony-forming unit (CFU) growth after GM-CSF or IL-3 stimulation. The response to growth factors acting via a growth factor receptor distinct from the GM-CSF/IL-3/IL-5 system (recombinant human granulocyte colony-stimulating factor [rhG-CSF]) was normal. After antileukemic treatment, the pulmonary symptoms resolved and betac or betac plus GM-CSFR alpha expression was normal. Our findings provide evidence that a defect in the expression of betac or betac plus GM-CSFR alpha on AML blasts can be associated with respiratory failure in patients with AML.

Acute Disease↗
PubMed1998

Hyperthermia for the treatment of patients with malignant germ cell tumors: a phase I/II study in ten children and adolescents with recurrent or refractory tumors.

BACKGROUND: Extracranial nontesticular germ cell tumors (GCTs) are rare malignancies in children and adolescents. Cisplatin-containing regimens and complete tumor resection are important determinants for a favorable outcome; however, patients with recurrent tumors that cannot be eradicated by surgical procedures and chemotherapy have a poor prognosis. Noninvasive electromagnetic technologies for superficial and regional deep hyperthermia (RHT) are under investigation to enhance local tumor control in various malignancies. The objectives of this Phase I/II study were to examine 1) whether RHT can be used in combination with platinum-based chemotherapy with acceptable toxicity in children and adolescents and 2) whether this combined regimen can induce objective tumor response in patients with malignant nontesticular GCT that persisted or recurred locoregionally after validated, intensive, cisplatin-based chemotherapy +/- surgery as unsuccessful first-line treatment. METHODS: The authors studied the effects of RHT induced by electromagnetic waves in combination with platinum-based chemotherapy in ten children and adolescents with recurrent or refractory GCTs. RESULTS: Seven of ten patients with recurrent or refractory GCTs had objective responses. Of these, two patients had a partial response and five patients had a complete response. CONCLUSIONS: The results of the current study found that combined RHT and platinum-based chemotherapy can be used in children and adolescents. This regimen was found to induce objective tumor response in 70% of study patients with recurrent or refractory GCTs. The results thus far are encouraging and the study has been extended to patients with a poor response to first-line treatment.

Adolescent↗
PubMed1998

Evaluation of follow-up investigations in osteosarcoma patients: suggestions for an effective follow-up program.

BACKGROUND AND PROCEDURE: Follow-up programs for cancer patients aim at improving the overall prognosis by early detection of relapse. In this study, follow-up data from 72 osteosarcoma patients were received in order to determine the value of clinical examination (CE), lung CT-scan (CTL), chest X-ray (CXR), local X-ray (LXR), and bone scintigraphy (BS) in the detection of tumor recurrence. PROCEDURE: Twenty-eight of 72 osteosarcoma patients presented with a total of 61 relapse sites. A continuous remission after relapse treatment could be achieved in 2/16 patients with first lung metastases, in 2/6 patients with local relapse, and in 3/19 patients with more than one lung metastasis. More than 90% of all relapses occurred within 3 years off primary therapy, respectively, within 3 years after detection of relapse. Local relapse and lung metastases were primarily diagnosed by CXR, CTL and CE. BS was the most important investigation to detect distant metastases. No relapse was found by routine X-ray of the primary tumor site. CONCLUSIONS: To improve efficacy of follow-up programs and to reduce radiation load of nonrelapsed patients, the prognosis of patients with lung metastases or local recurrences and the time of high risk for a relapse should be taken into consideration. Since the number of patients who benefit from relapse therapy is still low, it remains to be shown whether an increased frequency of lung CT-scans or MRIs of the primary tumor site will improve early detection of relapse; and if so, whether that will enhance the chance for successful relapse treatment. CXR, CTL and CE should be performed routinely for at least 3 years after completion of therapy or relapse diagnosis. In contrast, BS and LXR appear not to be useful as routine investigations.

Bone Neoplasms↗
PubMed1998

Teratomas in infancy and childhood.

BACKGROUND AND PROCEDURE: Outcomes in children with teratomas collected between October 1982 and December 1995 in cooperative protocols of the German Society of Pediatric Oncology and Hematology (GPOH) were analyzed. Teratomas were diagnosed in 329 (42%) of 780 registered patients with germ cell tumors. The annual incidence was 0.24/100,000. Main primary sites were coccygeal (n = 132, 2.2:1 female predominance), ovary (n = 81), testis (n = 40) and brain (n = 15, 2.8:1 male predominance). RESULTS: Two hundred seventy cases of extracranial non-testicular teratoma were evaluated: In mature teratomas (n = 154) the observed relapse rate was 10%. Incomplete resection was the main risk factor for relapse. After complete resection, the relapse-free survival (RFS, Kaplan-Meier-estimation) was 0.96 +/- 0.01 (n = 126, observation time 18-155 months) in comparison to an RFS of 0.56 +/- 0.09 in incompletely resected teratomas grade 0 (n = 28, observation time 28-94 months) (P < 0.01). Im-mature teratomas were treated by surgery alone in 76 cases and by surgery and adjuvant chemotherapy in 40 cases. The observed relapse risk was 18%. Main risk factors for relapse were incomplete tumor resection (n = 38) as well as immaturity in incompletely resected teratomas. Fifteen of 29 relapsing patients presented with malignant tissue in the recurrent tumor (mainly yolk sac tumor); in contrast, seven of 40 patients with immature teratoma relapsed despite adjuvant chemotherapy without showing malignant components (P = 0.014). Nine of 36 (25%) relapsing patients died of disease. Eleven of the 27 (41%) surviving children suffered from mutilation after repeated surgery. COMMENTS: It is suggested that an international randomized trial for patients with incompletely resected high risk teratoma be initiated to evaluate the effect of adjuvant chemotherapy on specific end-points: 1) influence on relapse rate in general; 2) reduction of the proportion of malignant relapses; 3) avoidance of mutilating surgery.

Adolescent↗
PubMed1998

Induction of a CD3+/CD56+ lymphocyte population following gene therapy with transgenic IL-2 secreting fibroblasts in a child with peripheral neuroectodermal malignancy.

BACKGROUND: Adjuvant interleukin-2 (IL-2) therapy after stem cell transplantation can improve the prognosis of patients with Ewing tumors. This has been attributed to stimulation of the immune system and its antineoplastic activity, thus eliminating minimal residual disease. As the side effects of systemic IL-2 limit the dosage, attempts have been made to locally augment the concentration of IL-2 in the proximity of the tumor. To achieve this, fibroblasts and/or tumor cells can be genetically modified to secrete IL-2 and then be injected to generate tumor immunogen. PROCEDURE: In a preliminary clinical trial we assessed whether the administration of transgenic IL-2-secreting fibroblasts was feasible without major toxicity and whether it had any effect regarding the activation of the immune system. We treated an 11-year-old boy with a peripheral neuroectodermal tumor of the left neck in fourth relapse, who was refractory to all available therapy. We transfected fibroblasts of the patient with an IL-2 gene expression vector using a cationic liposome reagent. In 51Cr cytotoxicity assays we obtained lysis of this patient's tumor cells by IL-2-stimulated mononuclear cells (MNCs). Under CT-guidance we intratumorally injected IL-2 transgenic autologous fibroblasts. RESULTS: We observed no local or systemic toxicity. In addition, we found a rise in the CD3+CD56+ lymphocyte population, previously described as cytokine-induced killer cells. No other hematological parameter changed significantly. CONCLUSIONS: Our data suggest that the intratumoral injection of transgenic IL-2-secreting fibroblasts is feasible without major toxicity and may lead to an increase in CD3+CD56+ cells.

CD3 Complex↗
PubMed1998

Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study.

OBJECTIVE: There is growing concern about the toxic side effects of daily oral cyclophosphamide (CYC) treatment. Intravenous (i.v.) pulse administration of CYC has been shown to be effective in patients with systemic lupus erythematosus, but contradictory results have been reported in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. METHODS: The efficacy and toxicity of i.v. pulse administration of CYC (0.75 gm/m2) versus daily oral CYC treatment (2 mg/kg body weight) were investigated in a prospective, randomized, multicenter study in patients with ANCA-associated vasculitis and renal involvement. RESULTS: The cumulative CYC dose was reduced by 57% in patients with i.v. pulse treatment (n = 22) compared with patients treated with daily oral therapy (n = 25). Patient survival, remission rate, time of remission, relapse rate, and outcome of renal function were not different between the 2 treatment groups. However, the rate of leukopenia (P < 0.01) and severe infections (P < 0.05 by 1-tailed test) was significantly reduced in the i.v. pulse group compared with the group receiving daily oral treatment. Moreover, gonadal toxicity was reduced in the i.v. pulse group, as indicated by significantly lower levels of follicle-stimulating hormone. CONCLUSION: This randomized study shows that i.v. CYC administration is an effective therapeutic tool with low toxicity in patients with ANCA-associated vasculitis and renal involvement.

Administration, Oral↗
PubMed1998

Endothelial dysfunction after bone marrow transplantation: increase of soluble thrombomodulin and PAI-1 in patients with multiple transplant-related complications.

Multiple transplant-related complications (MTRC) represent a severe condition after bone marrow transplantation (BMT) and are supposed to reflect systemic endothelial damage. Soluble thrombomodulin (sTM) and plasminogen activator inhibitor type-1 (PAI-1) were investigated as markers of endothelial dysfunction in 35 patients after autologous or allogeneic BMT and compared with the occurrence of the typical complications sepsis, veno-occlusive disease of the liver (VOD), graft-versus-host disease (GVHD), and capillary leakage syndrome (CLS). PAI-1 was assessed by an assay of functional activity and sTM by antigenic determination. In patients who had undergone allogeneic BMT and had no transplant-related complications (TRC), PAI-1 peaked on day +14 (20 +/- 5 units/ml), and sTM doubled in comparison to the starting range, to 60-80 ng/ml between days +14 and +49. In contrast, PAI-1 and sTM were unchanged following autologous BMT. PAI-1 was increased in sepsis, CLS, and VOD to 39-49 units/ml (p < 0.05, compared with patients without TRC), and in GVHD to 16-47 units/ml (not significant). Soluble TM increased to 63-309 ng/ml in patients with sepsis, VOD, or CLS (p < 0.05, compared with patients without TRC) and to 79-224 ng/ml in GVHD (not significant). The increase of sTM and PAI-1 was also positively correlated to the number of complications, so that in patients with three complications PAI-1 was increased 2.8-fold and sTM 3.5-fold over patients with no complications at all. We conclude that endothelial dysfunction is a feature of VOD, sepsis, CLS, and to lesser extent of GVHD and is worse in patients with multiple complications.

Adolescent↗
PubMed1998

Spontaneous splenic hemorrhage in a patient with Wegener's granulomatosis.

We report the occurrence of spontaneous splenic hemorrhage in a patient with Wegener's granulomatosis. Pulmonary infiltrates, hemoptysis, and crescentic glomerulonephritis were accompanied by a progressive splenic enlargement with minimal abdominal symptoms. Magnetic resonance imaging was particularly helpful. The spleen was removed by minimally invasive endoscopic surgery. Subcapsular hemorrhage had occurred because of splenic vasculitis. Postoperatively, a remission was achieved by a combination of high-dose corticosteroids and cyclophosphamide.

Endoscopy↗
PubMed1998

Hepatic veno-occlusive disease with severe capillary leakage after peripheral stem cell transplantation: treatment with recombinant plasminogen activator and C1-esterase inhibitor concentrate.

Severe veno-occlusive disease (VOD), characterised by elevated serum bilirubin levels, is a known complication in the first 3 weeks after peripheral blood stem cell transplantation (PBSCT). Severe VOD is associated with capillary leakage and multiple organ dysfunction and leads to high mortality. We report a 17-year-old male, who developed VOD with capillary leakage (CL) after allogeneic PBSCT. The patient presented with a maximum serum bilirubin of 25.4 mg/dl, weight gain (10% of baseline weight), generalized edema, cardiovascular insufficiency, complement activation, jaundice and a decreased AT and protein C functional activity. After VOD and CL were diagnosed the patient was treated with recombinant human plasminogen activator (rt-PA) and C1 esterase-inhibitor concentrate (C1-INH-C). The clinical symptoms resolved and the patient's status stabilized. The patient was in an adequate clinical state 5 months after transplantation. We noted that the combined therapy with rt-PA and C1-INH-C in this high-risk situation led to a resolution of VOD with CL.

Adolescent↗
PubMed1998

Combined therapy of medulloblastoma: review of 46 patients treated in a single institution.

Medulloblastoma is the most frequent malignant brain tumor in pediatric patients. Early treatment strategies, combining surgery and radiotherapy alone, resulted in survival rates of about 40% only. In the last 15 years, chemotherapy was used more frequently in combination with surgery and radiotherapy. The rationale was to increase the survival rate and to decrease radiation toxicity in young children. Forty-six patients younger than 16 years were treated between July 1977 and September 1995 in our institution (32 boys and 14 girls). Thirty-nine patients could be evaluated according to their postoperative treatment with regard to different protocols such as SIOP 1, SIOP 2, HDMTX/VCR, HIT protocol 89/91 and Carbo-PEI (one patient). In total, 21/39 patients are alive without evidence of disease (EFS 55 +/- 7%) with a follow-up of 26 to 210 months. Four children are lost to follow-up. Twelve patients died after relapse, 1 child died of MTX-induced brain atrophy, 1 patient developed a secondary malignancy (acute lymphoblastic leukemia) and died. The best results were seen in patients treated according to the HIT 89/91 protocol as first-line treatment (CR 9/10). The improvement in outcome of our patients with medulloblastoma in recent years suggests the benefit of intensified chemotherapy on survival. In addition, refinement in surgical and radiological treatment have certainly also contributed to the better results.

Adolescent↗
PubMed1998

Paraneoplastic limbic encephalitis in two teenage girls.

Paraneoplastic neurological disorders represent remote effects of cancer without invasion of tumor cells into the nervous system. Limbic encephalitis is a distinct entity mostly associated with small-cell carcinoma of the lung. We present the cases of two teenage girls who were admitted with clinical symptoms typical for limbic encephalitis. In the course of the disease, they exhibited characteristic evolutionary changes of brain MRI abnormalities. Onset of neurological symptoms and type of underlying neoplasia were different in both patients. In one girl the initial workup led to the diagnosis of nodular sclerosing Hodgkin disease which so far had not caused any symptoms besides the described neurological abnormalities. A diagnostic brain biopsy showed inflammatory changes and excluded invasion of malignant cells into the central nervous system. The other patient had been diagnosed with a small cell carcinoma of the ovary several months before neurological and brain MRI abnormalities were observed. This is the first report in which clinical picture, evolution of MRI abnormalities, and--in one case--characteristic neuropathological changes are suggestive of paraneoplastic limbic encephalitis in two adolescent girls.

Adolescent↗
PubMed1998

Primitive neuroectodermal tumors of the cerebral hemispheres in two siblings with TP53 germline mutation.

AWe report on two siblings (brother and sister) who developed cerebral PNETs at the age of 5 years and 6 months, respectively. Both children were treated by operation followed by polychemotherapy. The brother also received cranio-spinal irradiation. Nevertheless, the children died about 12 months and 24 months post-operatively due to extensive cerebral tumor recurrences. Shortly after having lost both of her children, the mother developed an intra-abdominal tumor, which was resected and histologically diagnosed as ovarian carcinoma. Because of this unusual familial clustering of tumors and a positive history of brain tumors and other cancers in several maternal relatives, we analyzed DNA isolated from both PNETs and the ovarian carcinoma as well as constitutional (leukocyte) DNA from the whole family for mutation of the TP53 tumor suppressor gene. This analysis revealed that all tumors were homozygous for a missense mutation at codon 213 (CGA => TGG) resulting in an amino acid exchange from arginine to tryptophane. The same mutation was present in one TP53 allele in the constitutional DNA of the mother and the children, indicating that the mother had transmitted a TP53 germline mutation to both of her children. Analysis of loss of heterozygosity at microsatellite markers from 17p confirmed deletion of the paternal (wild-type) allele in both PNETs. Further investigation of the PNETs by comparative genomic hybridization revealed multiple chromosomal abnormalities. Interestingly, some genomic changes were common to both PNETs, while many others were not, a finding suggesting substantial genomic instability, probably as a consequence of p53 inactivation.

Adult↗
PubMed1998

Complex chromosomal rearrangements associated with congenital erythrophagocytotic histiocytosis.

We describe a patient with a congenital malignant blood disorder and a constitutional de novo chromosomal rearrangement that includes four breakpoints. By conventional cytogenetic analysis an obviously reciprocal balanced translocation with the breakpoints 1p36 and 5q11.2 was diagnosed. Due to a suspicious dark band in the breakpoint area of 1p a more detailed analysis of the breakpoints was performed using microdissection and reverse chromosome painting. This revealed a small inversion at 1p36 that must have occurred prior to the reciprocal translocation. The three breakpoints in chromosome 1 (1p36.11, 1p36.21 and 1p36.31) are within or close by regions known to contain tumor suppressor genes. The chromosomal rearrangement might have resulted either in a submicroscopic deletion, in loss of heterozygosity of one or more imprinted genes, or in gene position effects as possible explanations for the clinical course of our patient.

Chromosomes, Human, Pair 1↗
PubMed1998

Management of severe chronic thrombocytopenia in von Willebrand's disease type 2B.

Two patients with a long history of unexplained thrombocytopenia, eventually diagnosed with von Willebrand's disease (vWD) type 2B are reported. In one patient with platelet counts of 80 x 10(9)/l 1-desamino-8-D-arginine vasopressin (DDAVP) had a favourable effect during bleeding episodes. The second patient received intermediate purity von Willebrand's factor (vWF)/factor VIII concentrate (Haemate HS), which helped haemostasis during tooth extraction. It increased platelet counts from 15 to 30 x 10(9)/l, whereas platelet transfusions produced no increase, nor prevented severe bleeding during abdominal surgery. Thus the treatment of vWD type 2B might depend on the degree of thrombocytopenia. It is recommended that in patients with mild to moderately decreased platelet counts, DDAVP treatment can be tried, whereas in patients with severely decreased platelet counts intermediate purity vWF/factor VIII concentrate substitution is preferred. In addition, vWD type 2B should be considered in the differential diagnosis of any child with chronic thrombocytopenia as the treatment strategy is different.

Adolescent↗