Iris Wieser

Lymphomatoid papulosis: Treatment response and associated lymphomas in a study of 180 patients

BACKGROUND Lymphomatoid papulosis (LyP) is a CD30(+) lymphoproliferative disorder, with a self-regressing clinical course and malignant histopathology. OBJECTIVE The aim of this study was to evaluate characteristics, risk factors, associated malignancies, long-term outcome, and treatment of LyP in a large cohort representing the experience of the MD Anderson Cancer Center. METHODS Patient charts and clinical and histopathologic data of 180 patients with LyP were retrospectively assessed. RESULTS A total of 56.7% of patients was men. Histologic subtype A was found in 47.2%, type B in 17.2%, type C in 22.8%, type D in 7.8%, type E in 0.6%, and mixed subtype in 4.4% of the patients. One hundred fourteen lymphomas were observed in 93 patients, with mycosis fungoides (61.4%) and anaplastic large cell lymphoma (26.3%) being the most common forms. Risk factors for development of lymphoma included sex and histologic subtype. Number of lesions and symptom severity were not associated with lymphoma development. Patients with type D were less likely to have lymphomas. Treatment provided symptomatic relief but did not prevent progression to lymphoma. LIMITATIONS The limitation of this study is the retrospective study design. CONCLUSION Patients with LyP are at increased risk of associated lymphomas. Thorough patient counseling is needed and long follow-up periods are required to detect and treat secondary lymphomas.

Tissue Doppler imaging in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome before and after fetal aortic valvuloplasty

Fetal aortic valvuloplasty can improve filling and reduce afterload of the left ventricle in critical aortic stenosis. Success of an intrauterine intervention is currently measured by technical success, clinical survival and eventual postnatal biventricular physiology. In the present study we investigated the use of tissue Doppler imaging (TDI) to evaluate changes in ventricular function assessed before and after prenatal aortic valvuloplasty.

Primär kutane CD30(+) lymphoproliferative Erkrankungen.

Primär kutane CD30+ lymphoproliferative Erkrankungen zählen zu der zweit häufigsten Gruppe der kutanen T‐Zell‐Lymphome (CTCL) und umfassen die Krankheitsbilder der lymphomatoiden Papulose (LyP) und des primär kutanen anaplastischen großzelligen Lymphoms (cALCL). Beide Erkrankungen haben klinische, histopathologische und molekulare Gemeinsamkeiten und repräsentieren ein Spektrum von kutanen CD30+ lymphoproliferativen Erkrankungen. Man kann LyP vom cALCL anhand des Zusammenspiels von klinischen und histopathologischen Befunden unterscheiden. In manchen Patienten können LyP und MF gemeinsam auftreten, oder können während des Krankheitsverlaufes entstehen. Mycosis fungoides (MF), ist die häufigste Form von CTCL und zählt nicht zur Gruppe der primär kutanen CD30+ lymphoproliferativen Erkrankungen. Manche LyP‐Patienten können jedoch von beiden Krankheitsbildern gemeinsam betroffen sein. Es ist aber auch möglich, dass ein MF‐Patient LyP‐artige Läsionen entwickelt, die eher eine Manifestation der MF darstellen als zwei unterschiedliche Erkrankungen. Besondere Vorsicht ist jedoch im Zusammenhang mit CD30+ transformierten MF‐Läsionen geboten, da die Gefahr besteht, dass diese fälschlicherweise als LyP oder cALCL diagnostiziert werden, was möglicherweise zu einer inadäquaten Behandlung führt.

Primary cutaneous CD30(+) lymphoproliferative disorders.

Primary cutaneous CD30+ lymphoproliferative disorders are the second most common group of cutaneous T‐cell lymphomas (CTCL) and include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large T‐cell lymphoma (cALCL). Both disease entities share overlapping clinical, histopathological, and molecular features, thus representing a spectrum of cutaneous CD30+ lymphoproliferative disorders. LyP may be distinguished from cALCL by clinicopathological correlation. In some patients, both diseases may coexist at initial diagnosis or develop over the course of the disease. Mycosis fungoides (MF), the most common CTCL, is not considered a primary cutaneous CD30+ lymphoproliferative disorder, but may occur in some LyP patients. In addition, LyP‐like lesions may develop in MF patients. However, this is frequently a manifestation of MF rather than a representation of two different disease entities. Caution also has to be taken in the setting of transformed MF with lesions expressing CD30, as they may be mistaken for either LyP or cALCL, resulting in an inadequate therapeutic approach.

Changes in ductus venosus flow profile in twin–twin transfusion syndrome: role in risk stratification

To evaluate changes in ductus venosus (DV) waveforms and the timing of these changes in twin–twin transfusion syndrome (TTTS), to relate these to disease severity and to assess the clinical applicability of the suggested measurements in the prediction of TTTS.

Ductus venosus alterations in twin‐twin transfusion syndrome: A useful tool for risk stratification

To evaluate changes in ductus venosus (DV) waveforms and the timing of these changes in twin–twin transfusion syndrome (TTTS), to relate these to disease severity and to assess the clinical applicability of the suggested measurements in the prediction of TTTS.

Cutaneous manifestations in trisomy 13 mosaicism: A rare case and review of the literature

Trisomy 13 mosaicism is a rare genetic disorder affecting a small minority of all trisomy 13 cases. It occurs when two cell populations that are karyotypically different are present in the same individual and are derived from a single zygote. As a rule, the phenotype is mitigated to a less dysmorphic appearance and longer survival, making genetic counseling a difficult task. Capillary hemangiomas are a common feature of full trisomy 13, seen in 27–56% of all cases. We report on an 18‐months‐old girl with extensive cutaneous anomalies, mild dysmorphic features, and slight psychomotor delay, without structural defects and provide an up‐to‐date review of all cases of trisomy 13 mosaicism with skin involvement. To our knowledge, this is the second clinical report of a patient with trisomy 13 mosaicism with hemangiomas and port wine stains, but no structural defects.