Immune Thrombocytopenia: A Rare Presentation of Pulmonary Sarcoidosis

Leal, Mariana da Silva; Amado, Carolina; Paracana, Bárbara; Pereira, Flávio; Sousa, Mariana Pacheco de; Mendes, Aurora

doi:10.12890/2021_003060

Cited by 4 publications

(5 citation statements)

References 6 publications

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“…While the association of thrombocytopenia with sarcoidosis is well documented, the presentation in practice is rare. Due to the rarity, there are no specific guidelines for the management of thrombocytopenia in patients with sarcoidosis [ 1 ]. Immune suppression with steroids is deemed the first-line treatment of thrombocytopenia with concurrent sarcoidosis [ 1 ] as steroids inhibit established autoimmunity and decrease granuloma formation in the bone marrow [ 12 ], which may enhance platelet count.…”

Section: Discussionmentioning

confidence: 99%

“…Due to the rarity, there are no specific guidelines for the management of thrombocytopenia in patients with sarcoidosis [ 1 ]. Immune suppression with steroids is deemed the first-line treatment of thrombocytopenia with concurrent sarcoidosis [ 1 ] as steroids inhibit established autoimmunity and decrease granuloma formation in the bone marrow [ 12 ], which may enhance platelet count. Furthermore, immune suppression is often utilized in the treatment of both thrombocytopenia and sarcoidosis as both are associated with an autoimmune or inflammatory state, thus providing the dual-utility of the treatment regimen in a patient with ITP secondary to sarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

“…Sarcoidosis is a multisystem disorder characterized by the formation of non-caseating granulomas [ 1 ]. It affects multiple organ systems including, but not limited to, the lungs, joints, heart, eyes, and skin [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…Isolated thrombocytopenia is an uncommon presentation of sarcoidosis compared to other hematological abnormalities, such as anemia and leukopenia [ 1 ]. Three main pathophysiological hypotheses of thrombocytopenia in sarcoidosis are proposed: hypersplenism, bone marrow infiltration of granulomas, and immune thrombocytopenia (ITP) [ 5 - 7 ].…”

Section: Introductionmentioning

confidence: 99%

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Severe Thrombocytopenia in a 30-Year-Old African American Male With Newly Diagnosed Sarcoidosis: A Case Report

Pasli¹,

Lovell²,

Vulasala³

et al. 2023

Cureus

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Sarcoidosis is a multisystem inflammatory disorder characterized by the formation of non-caseating granulomas. Hematological manifestations such as thrombocytopenia are unusual presentations of the disease. Various theories have been proposed for the development of thrombocytopenia in patients with sarcoidosis such as decreased production in bone marrow caused by granuloma formation, hypersplenism, and immune thrombocytopenia (ITP). We present a case of a 30-year-old African American male with ITP secondary to sarcoidosis who presented with a sudden onset of buccal mucosa and mucocutaneous bleeding and was found to have severe thrombocytopenia with values reaching as low as 1000/uL without prior history of easy bruising or bleeding. Overall, our patient had dyspnea, mucocutaneous bleeding, and was found to have mediastinal and hilar adenopathy, isolated thrombocytopenia, no splenomegaly, and nonnecrotizing granulomas in the lymph nodes. The patient received platelet transfusions without initial response and received intravenous immunoglobulin (IVIG), romiplostim, and steroids with subsequent improvement in the platelet count after sufficient administration of a treatment regimen of approximately one week. Confounding factors that resulted in diagnostic uncertainty of our patient presentation included travel history with prophylactic antimalarial medications, doxycycline usage, only slightly elevated Angiotensin-Converting Enzyme (ACE) levels, and imaging features concerning metastatic disease vs. lymphoma. The clinical diversity of sarcoidosis often leads to diagnostic uncertainty and treatment delays due to its resemblance to other more common disorders. This is a novel case report of the earliest temporal presentation of severe thrombocytopenia and sarcoidosis in an African American male reported in the literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Severe Thrombocytopenia in a 30-Year-Old African American Male With Newly Diagnosed Sarcoidosis: A Case Report

Pasli¹,

Lovell²,

Vulasala³

et al. 2023

Cureus

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“…Hematological manifestations such as thrombocytopenia are rare extrapulmonary complications (<1%) and have been rarely reported in sarcoidosis cases. This condition could be severe but usually follows a favorable course without death or severe bleeding [ 4 ]. Intracranial hemorrhage (ICH) is a rare and serious adverse effect of severe thrombocytopenia that can cause death or significant morbidity [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

A Rare Case of Relapsed Sarcoidosis Presenting As Severe Thrombocytopenia Associated With Intracerebral Hemorrhage Due to Bone Marrow Involvement

Weeraddana¹,

Othman²,

Thomas³

et al. 2023

Cureus

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Sarcoidosis is a systemic granulomatous disease characterized by the hyperactivation of CD4 T cells, CD8 T cells, and macrophages. Clinical presentations of sarcoidosis are highly variable. Sarcoidosis is unknown in its etiology, but it suggests it may result from exposure to specific environmental agents in genetically susceptible people. Sarcoidosis commonly involves the lungs and lymphoid system. Bone marrow involvement in sarcoidosis is uncommon. Sarcoidosis rarely results in intracerebral hemorrhage due to severe thrombocytopenia secondary to bone marrow involvement. We present the case of a 72-year-old woman who has been in remission from sarcoidosis for 15 years and developed intracerebral hemorrhage secondary to severe thrombocytopenia due to sarcoidosis recurrence in the bone marrow. The patient presented to the emergency department with a generalized, non-blanching petechiae rash and nose and gum bleeding. Her labs showed a platelet count of less than 10.000/mcL, and computed tomography (CT) showed intracerebral hemorrhage. A bone marrow biopsy revealed a small, non-caseating granuloma indicative of a sarcoidosis relapse in the bone marrow.

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Imaging findings in a rare case of alveolar, hepatic and splenic sarcoidosis presenting with thrombotic thrombocytopenic purpura

Vaccarino,

Parillo,

Perillo

et al. 2024

J Med Imaging Intervent Radiol

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Thrombotic thrombocytopenic purpura (TTP) is an acute haematological disorder characterized by severe ADAMTS13 enzyme deficiency, leading to consumptive thrombocytopenia, mechanical haemolysis, and organ damage. Its association with multisystemic sarcoidosis is extremely rare and, to the best of our knowledge, described in only two cases so far. We present the case of a 52-year-old woman with TTP and antibodies anti-ADAMTS13, showing computed tomography (CT) and magnetic resonance imaging (MRI) evidence of pulmonary, hepatic, and splenic lesions initially reported as ischemic/inflammatory changes. A follow-up MRI of the abdomen revealed increased evidence of the liver lesions, focal intrahepatic bile duct dilatation, splenic lesions, and enlarged hepatic hilar nodes. The follow-up chest CT showed increased evidence of the parenchymal lung consolidations. Given the radiological persistence of those alterations and the history of TTP, the hypothesis of an IgG4-related disease was then made. The IgG4 levels were found to be normal, while the histological examination of the liver revealed non-necrotizing granulomatous chronic inflammation. Elevated levels of angiotensin-converting enzyme were found, and the QuantiFERON-TB Gold test was negative for tuberculosis infection. Thus, the overall clinical picture was consistent with multisystemic sarcoidosis (alveolar, hepatic, and splenic). The diagnosis of sarcoidosis, already challenging due to the variability of its clinical presentation, can become even more complicated when it manifests with uncommon haematological manifestations such as TTP, along with non-specific extra-pulmonary involvement. While imaging aids in documenting organ damage, the definitive diagnosis of sarcoidosis necessitates histologic confirmation of noncaseating granulomas and the exclusion of other possible granulomatous diseases.

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Immune Thrombocytopenia: A Rare Presentation of Pulmonary Sarcoidosis

Cited by 4 publications

References 6 publications

Severe Thrombocytopenia in a 30-Year-Old African American Male With Newly Diagnosed Sarcoidosis: A Case Report

Severe Thrombocytopenia in a 30-Year-Old African American Male With Newly Diagnosed Sarcoidosis: A Case Report

A Rare Case of Relapsed Sarcoidosis Presenting As Severe Thrombocytopenia Associated With Intracerebral Hemorrhage Due to Bone Marrow Involvement

Imaging findings in a rare case of alveolar, hepatic and splenic sarcoidosis presenting with thrombotic thrombocytopenic purpura

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