Pulmonary veno-occlusive disease accompanied by microangiopathic hemolytic anemia 1 year after a second bone marrow transplantation for acute lymphoblastic leukemia.
Although hepatic veno-occlusive disease (HVOD) is a common complication of allogenic bone marrow transplantation (BMT), pulmonary veno-occlusive disease (PVOD) is very rare. Only three cases have been described in the literature. We report the case of a 19-year-old woman who developed PVOD accompanied by microangiopathic hemolytic anemia (MAHA) and hemolytic uremic syndrome (HUS) 1 year after a second BMT for relapsed acute lymphoblastic leukemia (ALL). Autopsy examination revealed obstruction of the small pulmonary veins with edematous thickening of the intima. These findings are compatible with PVOD. Pulmonary GVHD and pulmonary aspergillosis were also observed. Various etiologic factors have been implicated in PVOD after BMT. We postulate that pulmonary GVHD and pulmonary infection including aspergillosis played an important role in the occurrence of both PVOD and HUS in our patient. Microangiopathic cytokines released in response to the GVHD and infection may damage the intima of microvessels that were previously injured by the two BMT. Despite appropriate therapy, the microangiopathic process was irreversible and the patient died. Thus, measures must be taken to prevent and treat PVOD after BMT.
Kuga
T
T
Department of Internal Medicine, Japanese Red Cross Asahikawa Hospital, Japan.
Kohda
K
K
Hirayama
Y
Y
Matsumoto
S
S
Nakazawa
O
O
Ando
M
M
Ezoe
A
A
Nobuoka
A
A
Mochizuki
C
C
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000743:Anemia, Hemolytic / Q000209:etiology*; D000890:Anti-Infective Agents / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D001228:Aspergillosis / Q000150:complications / Q000188:drug therapy; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D003561:Cytarabine / Q000008:administration & dosage / Q000009:adverse effects; D017809:Fatal Outcome; D005260:Female; D006086:Graft vs Host Disease / Q000150:complications / Q000517:prevention & control; D006463:Hemolytic-Uremic Syndrome / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008172:Lung Diseases, Fungal / Q000150:complications / Q000188:drug therapy; D008942:Mitoxantrone / Q000008:administration & dosage / Q000009:adverse effects; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000628:therapy*; D011668:Pulmonary Veno-Occlusive Disease / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D019233:Retreatment; D019172:Transplantation Conditioning / Q000009:adverse effects
D000305:Adrenal Cortex Hormones; D000890:Anti-Infective Agents; D007166:Immunosuppressive Agents; D003561:Cytarabine; D008942:Mitoxantrone