Intravascular large B-cell lymphoma

Antemortem diagnosis with multiple random skin biopsies and transbronchial lung biopsy in a patient with
intravascular large B-cell lymphoma, the so-called Asian variant lymphoma

BMJ Case Reports 2014; doi:10.1136/bcr-2013-202661


Tomotaka Nishizawa, Takeshi Saraya, Haruyuki Ishii, Hajime Goto


Summary
A 59-year-old, previously healthy man presented to our hospital, with a 3-month history of high fever,
nocturnal sweating and exertional dyspnoea. Aggressive diagnostic procedures such as multiple random skin biopsies
and transbronchial lung biopsy (TBLB) led to an antemortem diagnosis of intravascular large B-cell lymphoma (IVLBCL),
which showed abundant CD20 atypical lymphocytes aggregated in lumina of small vessels. The 29 cases diagnosed with IVLBCL
during their lifetime by TBLB were reviewed. Their clinical features included respiratory symptoms
(hypoxaemia, dyspnoea and dry cough) and persistent fever. IVLBCL patients show various radiological patterns
(ground glass opacities, multiple centrilobular nodules, interlobular septal thickening, interstitial shadows
and thickening of bronchovascular bundles), suggesting lymphatic or haematological spread.
Antemortem diagnosis of IVLBCL is difficult, but a multidisciplinary approach, with aggressive multiple random skin biopsies
and/or TBLB, should be considered in patients with respiratory symptoms that are refractory to antibiotics or prednisolone treatment.

Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome

BMC Pulm Med. 2014 Mar 5;14(1):37. [Epub ahead of print]
Secondary pulmonary alveolar proteinosis complicating myelodysplastic syndrome results in worsening of prognosis: a retrospective cohort study in Japan.
Ishii H, Seymour JF, Tazawa R, Inoue Y, Uchida N, Nishida A, Kogure Y, Saraya T, Tomii K, Takada T, Itoh Y, Hojo M, Ichiwata T, Goto H, Nakata K.

Abstract

BACKGROUND:
Secondary pulmonary alveolar proteinosis (sPAP) is a very rare lung disorder comprising approximately 10% of cases of acquired PAP.
Hematological disorders are the most common underlying conditions of sPAP, of which 74% of cases demonstrate myelodysplastic syndrome (MDS).
However, the impact of sPAP on the prognosis of underlying MDS remains unknown.
The purpose of this study was to evaluate whether development of sPAP worsens the prognosis of MDS.

METHODS:
Thirty-one cases of sPAP and underlying MDS were retrospectively classified into mild and severe cases consisting of very low-/low-risk groups and
intermediate-/high-/very high-risk groups at the time of diagnosis of MDS,
according to the prognostic scoring system based on the World Health Organization classification.
Next, we compared the characteristics, disease duration, cumulative survival, and prognostic factors of the groups.

RESULTS:
In contrast to previous reports on the prognosis of MDS, we found that the cumulative survival probability for mild MDS patients
was similar to that in severe MDS patients. This is likely due to the poor prognosis of patients with mild MDS,
whose 2-year survival rate was 46.2%. Notably, 75% and 62.5% of patients who died developed fatal infectious diseases and exacerbation of PAP,
respectively, suggesting that the progression of PAP per se and/or PAP-associated infection contributed to poor prognosis.
The use of corticosteroid therapy and a diffusing capacity of the lung for carbon monoxide of less than 44% were predictive of poor prognosis.

CONCLUSION:
Development of sPAP during the course of MDS may be an important adverse risk factor in prognosis of patients with mild MDS.
PMID: 24597668 [PubMed - as supplied by publisher]