Asplenia means the absence of a spleen. Asplenia can occur in a variety of clinical settings, and it can refer to an anatomic absence of the spleen or functional asplenia secondary to a variety of disease states. The spleen is one of the primary extramedullary lymphoid organs. It is located in the left upper quadrant of the abdomen. Normally, the spleen is not palpable on physical exam and measures on average 10.65 by 5.16 cm. Normal spleen size can vary by gender and race. For example, men on average have larger spleens than females, and whites, on average, tend to have larger spleens compared to African American individuals.[1] When palpable or notably enlarged on imaging, the clinician must consider a variety of underlying disease states (whether acquired or inherited) contributing to the enlargement. Functionally, the spleen’s primary physiologic role is the filtration and processing of senescent blood cells (predominantly red blood cells or RBCs) and immunologically helps protect against encapsulated microorganisms and respond to infectious pathogens. It contains both hematopoietic and lymphopoietic elements, providing a basis for extramedullary hematopoiesis when necessary.[2] The spleen has two functionally and histologically distinct tissues where these processes take place: the white pulp and the red pulp. The white pulp has a large mass of lymphoid tissue that produces antibodies against recognized antigens, whereas the red pulp has a tight network of sinusoids called the cord of Billroth which helps in blood filtration.[3] As the body's largest filter of blood, it helps to remove old RBCs from the circulation aiding in the removal of blood-borne microorganisms.[4] Beyond the spleen, the lymphatic system of the body consists of a complex myriad of lymphatic vessels, lymph nodes, and other specific extramedullary lymphoid organs including the thymus, tonsils, and appendix, which collectively play a very important role in immune defenses. Asplenia can be caused by damage to the white pulp, the red pulp, or both. The spleen is a direct and indirect site of potential toxicity that can secondarily arise from underlying disease processes or infection.[2] One must understand and appreciate the various etiologies and potential complications of asplenia, such as being a great risk factor for encapsulated microorganisms. Bacterial infections with Neisseria meningitides or Streptococcal pneumoniae, among many others, can become life-threatening if untreated in patients with anatomic or functional asplenia.[5] Patients without a spleen have a 200-fold greater risk of death from septicemia compared to those with an adequate functioning spleen. There are many case reports in the medical literature reporting of asplenic patients, unfortunately, succumbing to infectious disease leading to death. One notable example reported by Heubner ML et al. (2015) described the rare case of a patient who had required splenectomy due to refractory immune thrombocytopenia (ITP).[6] He subsequently developed a pneumococcal meningitis infection and died despite best supportive efforts.  The rate of invasive bacterial infection and mortality in asplenic patients is significantly higher compared to normal controls – 69% in meningitis, 64% in septicemia, and 7% in Purpura fulminans.[5]

While widespread coronavirus disease 2019 (COVID-19) vaccination has helped achieve some control of the pandemic, vaccines have presented with side effects of their own, both common and rare. We present an unusual case of a 66-year-old who presented with severe thrombocytopenia following vaccination with the Pfizer-BioNTech mRNA vaccine. Our patient is a 66-year-old African American female with a known history of Sjogren's syndrome and hepatitis C who presented to our facility as a direct admit from our affiliated infusion clinic where routine lab work revealed a platelet count of 14,000. On arrival, she reported a one-month history of progressive tiredness, intermittent epistaxis, and bruising on her legs. Her physical exam was notable for multiple petechiae and non-palpable purpura on all four extremities. Further questioning revealed that she had received her COVID-19 vaccine booster (Pfizer-BioNTech) three weeks prior to presentation and that is when all the symptoms had started. Rheumatology was consulted and the patient was started on intravenous immunoglobulin infusion for two days and pulse dose prednisone. Her platelet count showed improvement after treatment, and she was discharged home with a platelet count of 42,000. Though largely safe and efficacious, COVID-19 vaccines can present with rare systemic side effects and physicians must have a high index of suspicion and report these cases so that more data is available for interpretation.

We present the case of a 10-year-old boy who was admitted with 3 months of episodic febrile neutropenia and a new petechial rash. Routine bloods identified neutropenia, thrombocytopenia, and a raised alanine aminotransferase. The dermatology team was consulted in light of the symmetrical petechial eruption of the upper torso. A punch biopsy of the lesion was consistent with early capillaritis. The results showed superficial dermal red blood cell extravasation with mild perivascular lymphohistiocytic inflammatory infiltrate. There was no evidence of an atypical lymphoid infiltrate in the skin biopsy. An initial bone marrow aspirate showed an abnormal mature T-cell population consisting of CD4 and CD8 T cells with gamma-delta positivity. Karyotyping was also done, which demonstrated isochromosome 7q. These findings were consistent with a diagnosis of hepatosplenic T-cell lymphoma (HSTL). The patient underwent fourth-line chemotherapy due to refractory relapsing disease but sadly passed away within 12 months of diagnosis. HSTL is a rare and aggressive subset of peripheral T-cell lymphoma. Prognosis is poor with a median survival of <1 year from diagnosis. However, reports suggest improved outcomes if intensive, early, high-dose chemotherapy is used alongside hematopoietic stem cell transplantation. Therefore, there is an impetus to attain early diagnosis for aggressive early treatment and improved patient outcomes. Capillaritis, presenting as asymptomatic nonpalpable purpura, can be a rare presenting feature of HSTL. Dermatologists could play a pivotal role in the early recognition of this rare but aggressive hematological malignancy and promote prompt treatment resulting in better patient outcomes.

The etiology of purpura in Sjögren syndrome (SS) includes cryoglobulinemia, hypergammaglobulinemia, and leukocytoclastic vasculitis (LCV). The clinical symptoms of LCV associated with SS comprise palpable or nonpalpable purpura and urticarial vasculitis. Here, we report a case of LCV presenting as erythema gyratum repens (EGR)-like purpura in a 62-year-old woman with rheumatoid arthritis and SS. EGR-like skin lesions, characterized by annular lesions with expanding concentric pattern and coalescing to form a zebra-like pattern or grain of wood pattern, can appear in various autoimmune conditions; however, EGR-like eruption in SS is extremely rare. On the basis of the expansion pattern, we considered that the EGR-like purpura in this case was elicited by urticarial vasculitis accompanied by SS.

Henoch-Schonlein purpura (HSP) is primarily a childhood immunoglobulin A (IgA)-mediated illness. When adults are affected, malignancy can be associated. We present a rare case of HSP in a 75-year-old man with malignant pleural mesothelioma. He presented with episodes of dizziness and subsequently developed non-palpable purpura across his legs, arthralgia, hematuria, proteinuria, and acute renal impairment. HSP was diagnosed based on clinical and histological findings on biopsy specimens from the skin and kidney that showed a leukocytoclastic vasculitis and mesangioproliferative glomerulonephritis with IgA deposits, respectively. He was treated with high-dose oral steroids with resolution of the skin and renal manifestations of the disease. HSP is rare in adults but has been linked to cancers. This is the first report of HSP in a patient with known malignant pleural mesothelioma.

Purpura annularis telangiectodes (PAT), also known as Majocchi purpura, is a rare form of pigmented purpuric dermatosis characterized by non-palpable red-brown, occasionally pruritic patches which progress to hyperpigmented halos. Purpura annularis telangiectodes usually presents in female adolescents as benign symmetric lesions with a predilection for the lower extremities. We present an atypical case of unilateral PAT in an elderly male. To our knowledge, our patient at 85-years-old is the oldest PAT and first unilateral purpura annularis telangiectodes case described in the literature.

Pigmented purpuric dermatosis (PPD) is a group of chronic disorders characterized by the extravasation of erythrocytes and marked hemosiderin deposits in the dermis. Schamberg disease is a subtype of pigmented purpuric dermatosis in which nonpalpable purpura is prominent on the lower extremities. No cases of primary antiphospholipid syndrome and pigmented purpuric dermatosis have so far been reported in the literature. We herein report a case of pigmented purpuric dermatosis associated with primary antiphospholipid syndrome.

Hepatitis B can have varied extrahepatic manifestations involving the skin, renal, haematological and nervous systems. Neurological manifestations in hepatitis B may take the form of Guillain-Barré syndrome and secondary systemic vasculitis-related mononeuritis multiplex. The clinical course of hepatitis B-related, vasculitis-related neuropathy is usually subacute to chronic and clinical evolution is relatively benign. To our knowledge, acute hepatitis B-associated vasculitis manifesting as acute distal symmetric polyneuropathy has not been reported. We report a 60-year-old man who presented with fever, mild hepatomegaly, skin lesions in the form of non-palpable purpura and acute onset distal symmetric sensorimotor polyneuropathy. Serum transaminase levels were raised and viral serological markers revealed acute hepatitis B. The patient remained anicteric throughout his clinical course. Nerve conduction studies showed severe axonal sensorimotor polyneuropathy and histopathological examination of sural nerve biopsy was suggestive of vasculitic neuropathy. The patient was first given a course of intravenous immunoglobulin with the antiviral drug entecavir. The fever subsided after 1 week of treatment. The patient was started on prednisolone in addition to the entecavir, and showed significant improvement in motor power and marked resolution in paresthesia after 2 weeks of treatment. Thus, acute onset distal symmetric sensorimotor polyneuropathy of vasculitic etiology can be a manifestation of acute hepatitis B.